Vogt-Koyanagi-Harada Disease

Autoimmune MONDO:0018092 Pathograph 14 Autoimmune Disease Ophthalmic Disorder

Vogt-Koyanagi-Harada disease is a multisystem autoimmune inflammatory disease targeting melanin-containing tissues, especially the uvea and choroid, with neurologic, auditory, skin, and hair involvement. Disease expression reflects immune susceptibility and inflammatory triggers, and early systemic immunosuppression is used to prevent chronic recurrent ocular disease.

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5
Pathophys.
10
Phenotypes
14
Pathograph
3
Genes
4
Treatments
2
Trials
9
References
1
Deep Research
⚙

Pathophysiology

5
Melanocyte-Directed Autoimmune Inflammation
Autoreactive immune responses against melanin-containing tissues drive VKH disease. The model links HLA-associated susceptibility and immune activation to inflammation of the uvea, meninges, ear, skin, and hair.
helper T cell link melanocyte link
adaptive immune response link ↑ INCREASED T cell activation link ↑ INCREASED
Downstream
Bilateral Granulomatous Panuveitis Autoimmune inflammation in melanocyte-rich uveal and choroidal tissues produces bilateral granulomatous ocular inflammation.
Extraocular Melanocyte-Containing Tissue Involvement The same autoimmune process affects meninges, inner ear, skin, and hair.
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
This review-level clinical synthesis supports the multisystem autoimmune disease model and the affected melanin-containing tissue distribution.
Bilateral Granulomatous Panuveitis
Ocular inflammation in VKH typically includes bilateral uveitis with diffuse choroidal thickening, subretinal fluid, and possible optic nerve involvement, which can progress to chronic recurrent inflammation and sunset glow fundus.
T cell link macrophage link
inflammatory response link ↑ INCREASED leukocyte migration link ↑ INCREASED
Downstream
Visual Impairment Ocular inflammation, subretinal fluid, and chronic recurrent complications can reduce visual acuity.
Show evidence (1 reference)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"This typically presents in the eye with acute findings of granulomatous anterior uveitis, diffuse choroidal thickening, multiple focal areas of sub-retinal fluid and, in severe cases, optic nerve involvement with bullous serous retinal detachment can occur."
The retrospective clinical series and review directly describes the ocular inflammatory features represented in this pathophysiology node.
Cytokine-Amplified Chronic Recurrent Inflammation
Immune risk loci and inflammatory cytokine production contribute to ongoing inflammatory activity. Chronic recurrent disease is associated with worse initial visual acuity, sunset glow fundus, ocular complications, and recurrent inflammation.
T cell link macrophage link
cytokine production link ↑ INCREASED tumor necrosis factor production link ↑ INCREASED
Downstream
Bilateral Granulomatous Panuveitis Persistent cytokine production sustains ocular inflammatory activity.
Show evidence (1 reference)
DOI:10.1186/s40246-023-00459-7 SUPPORT Human Clinical
"Functional studies on rs3812555 genotyped individuals revealed that CC carriers had significantly higher CARD9 mRNA expression and tumour necrosis factor-α production than TC/TT carriers (P = 1.00 × 10–4; P = 2.00 × 10–3, respectively)."
This human genetic association study links a VKH susceptibility genotype with higher CARD9 expression and TNF-alpha production.
Extraocular Melanocyte-Containing Tissue Involvement
VKH extends beyond ocular inflammation to melanocyte-containing tissues in the ears, brain or meninges, skin, and hair.
melanocyte link
inflammatory response link ↑ INCREASED
Downstream
Meningeal Irritation Extraocular inflammation can involve the meninges.
Sensorineural Hearing Loss Extraocular inflammation can involve the auditory system.
Vitiligo Melanocyte-directed inflammation can involve the skin.
Alopecia Melanocyte-directed inflammation can involve hair.
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
This review supports extraocular involvement across the auditory, neurologic, skin, and hair systems.
Visual Impairment
Acute and chronic recurrent ocular inflammation can reduce best-corrected visual acuity and contribute to persistent visual impairment.
inflammatory response link ↑ INCREASED
Show evidence (1 reference)
DOI:10.1186/s12886-024-03511-9 SUPPORT Human Clinical
"Initial best-corrected visual acuity (BCVA) examination at the first visit showed an average BCVA of 0.64 ± 0.29 logMAR in the acute-resolved group and 1.38 ± 0.54 logMAR in the chronic-recurrent group (p = 0.002)."
This retrospective VKH cohort links chronic recurrent disease with worse measured visual acuity.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Vogt-Koyanagi-Harada Disease Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
●

Phenotypes

10
Ear 2
Tinnitus Tinnitus (HP:0000360)
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
Ear involvement directly supports auditory manifestations such as tinnitus without relying on uncached full-text frequency data.
Sensorineural Hearing Loss Sensorineural hearing impairment (HP:0000407)
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
The review supports auditory system involvement; the broader HPO hearing impairment term is used because the abstract does not distinguish tinnitus from hearing loss.
Eye 1
Bilateral Panuveitis Panuveitis (HP:0012121)
Show evidence (1 reference)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease which targets melanin-containing tissues such as the uvea, meninges, ear and skin."
The abstract identifies the uvea as a target tissue, supporting uveitis as a core ocular phenotype.
Integument 1
Alopecia Alopecia (HP:0001596)
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
Hair involvement directly supports alopecia as an integumentary VKH manifestation without relying on uncached full-text frequency data.
Nervous System 1
Headache Headache (HP:0002315)
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
Brain involvement in this multisystem inflammatory disease supports headache as a neurologic manifestation without relying on uncached full-text frequency data.
Other 5
Serous Retinal Detachment Serous retinal detachment (HP:0012231)
Show evidence (1 reference)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"optic nerve involvement with bullous serous retinal detachment can occur."
The clinical review explicitly describes serous retinal detachment as an ocular finding in severe VKH.
Sunset Glow Fundus Fundus hypopigmentation (HP:0007894)
Show evidence (1 reference)
DOI:10.1186/s12886-024-03511-9 SUPPORT Human Clinical
"“Sunset glow fundus” was observed in 23.5% of the acute-resolved group and 64.3% of the chronic-recurrent group patients (P = 0.001)."
This VKH cohort directly documents sunset glow fundus and its enrichment in chronic recurrent disease. Fundus hypopigmentation is used as the closest validated local HPO binding for the depigmented fundus finding.
Meningeal Irritation Non-infectious meningitis (HP:0033430)
Show evidence (1 reference)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease which targets melanin-containing tissues such as the uvea, meninges, ear and skin."
Meningeal involvement supports the non-infectious meningitis or meningeal irritation component of VKH.
Vitiligo Vitiligo (HP:0001045)
Show evidence (1 reference)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease which targets melanin-containing tissues such as the uvea, meninges, ear and skin."
Skin involvement in a melanocyte-targeting disease supports vitiligo as an integumentary VKH phenotype.
Poliosis Poliosis (HP:0002290)
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease affecting the eyes, ears, brain, skin, and hair."
Hair involvement in VKH supports poliosis as a hair depigmentation manifestation.
🧬

Genetic Associations

3
HLA-DRB1*04:05 susceptibility (HLA-DRB1*04:05 is associated with susceptibility to VKH and VKH-like immune-related uveitis.)
Show evidence (1 reference)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Different possible pathogenetic mechanisms underlying the onset of VKH after COVID-19 vaccination are discussed, while the presence of the HLA DR4 antigen as a genetic predisposition for the onset of the disease after COVID-19 infection and vaccination is proposed."
The review supports HLA-DR4/HLA-DRB1-related genetic predisposition in triggered VKH contexts.
PRKCD susceptibility locus (PRKCD rs74437127 alleles are associated with altered VKH susceptibility in a Chinese Han case-control cohort.)
Show evidence (1 reference)
DOI:10.1186/s40246-023-00459-7 SUPPORT Human Clinical
"We found that rs74437127 C allele of PRKCD, rs3812555 CC genotype, and C allele of CARD9 were associated with increased susceptibility of VKH (Pc = 0.020, OR = 1.624; Pc = 2.04 × 10–5, OR = 1.810; Pc = 2.76 × 10–5, OR = 1.698, respectively)."
This human case-control study directly supports PRKCD as a susceptibility locus.
CARD9 susceptibility locus (CARD9 rs3812555 is associated with VKH susceptibility and increased inflammatory cytokine production.)
Show evidence (1 reference)
DOI:10.1186/s40246-023-00459-7 SUPPORT Human Clinical
"We found an association between PRKCD rs74437127 and CARD9 rs3812555 polymorphisms and VKH susceptibility and revealed that the increased susceptibility of rs3812555 for VKH may be mediated by regulating CARD9 gene expression and the production of pro-inflammatory cytokines, such as TNF-α."
This conclusion supports CARD9 as a susceptibility locus with a plausible cytokine-mediated functional correlate.
💊

Treatments

4
Early Systemic Corticosteroids
Action: Pharmacotherapy NCIT:C15986
Agent: corticosteroid ↗
High-dose systemic corticosteroids are used early to suppress acute ocular and systemic inflammation, commonly followed by early immunomodulatory therapy to reduce chronic progression risk.
Target Phenotypes: Panuveitis ↗
Show evidence (1 reference)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"Treatment is usually initiated with corticosteroids followed by an early introduction of immunosuppressive treatment (IMT) to achieve immediate response after disease presentation, although the choice of IMT for VKH can vary."
This clinical review supports systemic corticosteroids as the initial VKH treatment backbone.
Combined Immunomodulatory Therapy
Action: Pharmacotherapy NCIT:C15986
Agent: mycophenolate mofetil ↗ cyclosporin A ↗
Conventional steroid-sparing immunomodulatory therapy, including agents such as mycophenolate mofetil or cyclosporine, is used with low-dose steroids to stabilize disease and preserve vision.
Target Phenotypes: Panuveitis ↗
Show evidence (2 references)
DOI:10.1186/s12348-023-00333-6 SUPPORT Human Clinical
"81% (21 of 26 patients) of our patients treated with combined IMT/steroid were able to achieve disease stability with significant good visual outcome at 24 months (Median VApre-IMT = 0.3 Logmar vs VApost-IMT = 0.0 Logmar, p = 0.0001)."
This retrospective clinical series supports combined immunomodulatory therapy and low-dose steroid treatment for VKH disease stability.
DOI:10.1038/s41467-023-39483-5 SUPPORT Human Clinical
"Here, we report that combined with a non-standard corticosteroid regimen, cyclosporine-based immunosuppressant strategy is non-inferior to adalimumab-based biologic strategy by 26 weeks for visual improvement in a cohort of patients with Vogt-Koyanagi-Harada disease, 75% of whom have a..."
The randomized non-inferiority trial supports cyclosporine-based conventional immunosuppression as an evidence-backed therapeutic strategy.
Adalimumab Biologic Therapy
Action: Pharmacotherapy NCIT:C15986
Agent: adalimumab ↗
Adalimumab, an anti-TNF monoclonal antibody, is used for refractory or chronic recurrent VKH and is under study for acute VKH in combination with glucocorticoids.
Mechanism Target:
INHIBITS Cytokine-Amplified Chronic Recurrent Inflammation — Anti-TNF biologic therapy is represented as reducing cytokine-amplified chronic recurrent ocular inflammation.
Target Phenotypes: Panuveitis ↗
Show evidence (1 reference)
DOI:10.1186/s12886-024-03511-9 SUPPORT Human Clinical
"ADA significantly reduced anterior chamber inflammatory cells (P = 0.000) and vitreous cavity inflammatory cells (P = 0.001) in the chronic-recurrent group, and markedly decreased the recurrence rate in VKH patients (P = 0.009)."
This retrospective cohort directly supports adalimumab efficacy in chronic recurrent VKH.
Janus Kinase Inhibitor Therapy
Action: Pharmacotherapy NCIT:C15986
Agent: baricitinib ↗ tofacitinib ↗
JAK inhibitors are an emerging option for refractory non-infectious ocular inflammatory disease; available evidence includes a prospective registry cohort with one VKH patient, so this is promising but not VKH-specific definitive evidence.
Target Phenotypes: Panuveitis ↗
Show evidence (1 reference)
DOI:10.3389/fmed.2024.1439338 PARTIAL Human Clinical
"Ocular inflammation was related to a systemic disease in 8 (66.7%) patients as follows: spondyloarthritis (n = 3), peripheral psoriatic arthritis (n = 1), rheumatoid arthritis (n = 1), antinuclear antibodies (ANA) positive juvenile idiopathic arthritis (n = 1), Behçet’s syndrome (n = 1),..."
The prospective registry includes a VKH case among non-infectious ocular inflammatory diseases, so it partially supports JAK inhibitor relevance without establishing VKH-specific efficacy.
🌍

Environmental Factors

2
SARS-CoV-2 infection or vaccination
COVID-19 infection and SARS-CoV-2 vaccination have been reported before VKH onset or relapse. The association is treated as a trigger context rather than proof of a necessary cause.
Show evidence (2 references)
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"The Coronavirus Disease 2019 (COVID-19) is a new contagious infection that might trigger the onset of VKH disease, as previously proposed for other viruses."
The review supports SARS-CoV-2 infection as a reported possible trigger.
DOI:10.3390/jcm12196242 SUPPORT Human Clinical
"Moreover, after the mass vaccination against SARS-CoV-2 worldwide, cases of VKH disease associated with COVID-19 vaccination have been reported."
The review supports COVID-19 vaccination as a reported onset or relapse context.
Immune checkpoint inhibitor therapy
Immune checkpoint inhibitor therapy can be associated with VKH-like immune-related uveitis, especially in HLA-DRB1*04:05-positive patients.
Show evidence (1 reference)
DOI:10.1038/s41598-023-40565-z SUPPORT Human Clinical
"Statistical analysis showed that HLA-DRB1*04:05 was significantly associated with developing VKH-like ICIU (P = 0.029)."
This case-series abstract supports the reported HLA-DRB1*04:05-associated VKH-like immune checkpoint inhibitor uveitis context.
🔬

Clinical Trials

2
NCT03399175 NOT_APPLICABLE RECRUITING
Prospective study of early systemic high-dose corticosteroid and immunosuppressive therapy from VKH disease onset with multimodal clinical follow-up.
Target Phenotypes: Panuveitis ↗
Show evidence (1 reference)
clinicaltrials:NCT03399175 SUPPORT Human Clinical
"This prospective study will include patients with Vogt-Koyanagi-Harada disease from disease onset, treated with early systemic high-dose corticosteroid and immunosuppressive therapy."
ClinicalTrials.gov identifies this VKH study of early systemic corticosteroid plus immunosuppressive therapy.
NCT05590416 NOT_APPLICABLE RECRUITING
Observational study of adalimumab treatment in acute Vogt-Koyanagi-Harada disease.
Target Phenotypes: Panuveitis ↗
Show evidence (1 reference)
clinicaltrials:NCT05590416 SUPPORT Human Clinical
"This project is designed to test the hypothesis that adalimumab is clinically useful for patients with acuta Vogt-Koyanagi-Harada disease"
ClinicalTrials.gov identifies an acute VKH observational study of adalimumab.
{ }

Source YAML

click to show 
name: Vogt-Koyanagi-Harada Disease
creation_date: "2026-05-11T12:14:15Z"
updated_date: "2026-05-11T16:32:00Z"
category: Autoimmune
disease_term:
  preferred_term: Vogt-Koyanagi-Harada disease
  term:
    id: MONDO:0018092
    label: Vogt-Koyanagi-Harada disease
parents:
- Autoimmune Disease
- Ophthalmic Disorder
synonyms:
- VKH disease
- Vogt-Koyanagi-Harada syndrome
- Harada disease
description: >-
  Vogt-Koyanagi-Harada disease is a multisystem autoimmune inflammatory disease
  targeting melanin-containing tissues, especially the uvea and choroid, with
  neurologic, auditory, skin, and hair involvement. Disease expression reflects
  immune susceptibility and inflammatory triggers, and early systemic
  immunosuppression is used to prevent chronic recurrent ocular disease.
pathophysiology:
- name: Melanocyte-Directed Autoimmune Inflammation
  description: >-
    Autoreactive immune responses against melanin-containing tissues drive VKH
    disease. The model links HLA-associated susceptibility and immune activation
    to inflammation of the uvea, meninges, ear, skin, and hair.
  cell_types:
  - preferred_term: helper T cell
    term:
      id: CL:0000912
      label: helper T cell
  - preferred_term: melanocyte
    term:
      id: CL:0000148
      label: melanocyte
  biological_processes:
  - preferred_term: adaptive immune response
    term:
      id: GO:0002250
      label: adaptive immune response
    modifier: INCREASED
  - preferred_term: T cell activation
    term:
      id: GO:0042110
      label: T cell activation
    modifier: INCREASED
  downstream:
  - target: Bilateral Granulomatous Panuveitis
    description: >-
      Autoimmune inflammation in melanocyte-rich uveal and choroidal tissues
      produces bilateral granulomatous ocular inflammation.
  - target: Extraocular Melanocyte-Containing Tissue Involvement
    description: >-
      The same autoimmune process affects meninges, inner ear, skin, and hair.
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      This review-level clinical synthesis supports the multisystem autoimmune
      disease model and the affected melanin-containing tissue distribution.
- name: Bilateral Granulomatous Panuveitis
  description: >-
    Ocular inflammation in VKH typically includes bilateral uveitis with diffuse
    choroidal thickening, subretinal fluid, and possible optic nerve involvement,
    which can progress to chronic recurrent inflammation and sunset glow fundus.
  cell_types:
  - preferred_term: T cell
    term:
      id: CL:0000084
      label: T cell
  - preferred_term: macrophage
    term:
      id: CL:0000235
      label: macrophage
  biological_processes:
  - preferred_term: inflammatory response
    term:
      id: GO:0006954
      label: inflammatory response
    modifier: INCREASED
  - preferred_term: leukocyte migration
    term:
      id: GO:0050900
      label: leukocyte migration
    modifier: INCREASED
  downstream:
  - target: Visual Impairment
    description: >-
      Ocular inflammation, subretinal fluid, and chronic recurrent complications
      can reduce visual acuity.
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      This typically presents in the eye with acute findings of granulomatous
      anterior uveitis, diffuse choroidal thickening, multiple focal areas of
      sub-retinal fluid and, in severe cases, optic nerve involvement with
      bullous serous retinal detachment can occur.
    explanation: >-
      The retrospective clinical series and review directly describes the ocular
      inflammatory features represented in this pathophysiology node.
- name: Cytokine-Amplified Chronic Recurrent Inflammation
  description: >-
    Immune risk loci and inflammatory cytokine production contribute to ongoing
    inflammatory activity. Chronic recurrent disease is associated with worse
    initial visual acuity, sunset glow fundus, ocular complications, and
    recurrent inflammation.
  cell_types:
  - preferred_term: T cell
    term:
      id: CL:0000084
      label: T cell
  - preferred_term: macrophage
    term:
      id: CL:0000235
      label: macrophage
  biological_processes:
  - preferred_term: cytokine production
    term:
      id: GO:0001816
      label: cytokine production
    modifier: INCREASED
  - preferred_term: tumor necrosis factor production
    term:
      id: GO:0032640
      label: tumor necrosis factor production
    modifier: INCREASED
  downstream:
  - target: Bilateral Granulomatous Panuveitis
    description: >-
      Persistent cytokine production sustains ocular inflammatory activity.
  evidence:
  - reference: DOI:10.1186/s40246-023-00459-7
    reference_title: Genetic association of PRKCD and CARD9 polymorphisms with Vogt-Koyanagi-Harada disease in the Chinese Han population
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Functional studies on rs3812555 genotyped individuals revealed that CC
      carriers had significantly higher CARD9 mRNA expression and tumour
      necrosis factor-α production than TC/TT carriers (P = 1.00 × 10–4; P = 2.00 × 10–3, respectively).
    explanation: >-
      This human genetic association study links a VKH susceptibility genotype
      with higher CARD9 expression and TNF-alpha production.
- name: Extraocular Melanocyte-Containing Tissue Involvement
  description: >-
    VKH extends beyond ocular inflammation to melanocyte-containing tissues in
    the ears, brain or meninges, skin, and hair.
  cell_types:
  - preferred_term: melanocyte
    term:
      id: CL:0000148
      label: melanocyte
  biological_processes:
  - preferred_term: inflammatory response
    term:
      id: GO:0006954
      label: inflammatory response
    modifier: INCREASED
  downstream:
  - target: Meningeal Irritation
    description: Extraocular inflammation can involve the meninges.
  - target: Sensorineural Hearing Loss
    description: Extraocular inflammation can involve the auditory system.
  - target: Vitiligo
    description: Melanocyte-directed inflammation can involve the skin.
  - target: Alopecia
    description: Melanocyte-directed inflammation can involve hair.
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      This review supports extraocular involvement across the auditory,
      neurologic, skin, and hair systems.
- name: Visual Impairment
  description: >-
    Acute and chronic recurrent ocular inflammation can reduce best-corrected
    visual acuity and contribute to persistent visual impairment.
  biological_processes:
  - preferred_term: inflammatory response
    term:
      id: GO:0006954
      label: inflammatory response
    modifier: INCREASED
  evidence:
  - reference: DOI:10.1186/s12886-024-03511-9
    reference_title: Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Initial best-corrected visual acuity (BCVA) examination at the first visit
      showed an average BCVA of 0.64 ± 0.29 logMAR in the acute-resolved group
      and 1.38 ± 0.54 logMAR in the chronic-recurrent group (p = 0.002).
    explanation: >-
      This retrospective VKH cohort links chronic recurrent disease with worse
      measured visual acuity.
phenotypes:
- category: Ophthalmologic
  name: Bilateral Panuveitis
  diagnostic: true
  phenotype_term:
    preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease
      which targets melanin-containing tissues such as the uvea, meninges, ear
      and skin.
    explanation: >-
      The abstract identifies the uvea as a target tissue, supporting uveitis as
      a core ocular phenotype.
- category: Ophthalmologic
  name: Serous Retinal Detachment
  phenotype_term:
    preferred_term: Serous retinal detachment
    term:
      id: HP:0012231
      label: Serous retinal detachment
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      optic nerve involvement with bullous serous retinal detachment can occur.
    explanation: >-
      The clinical review explicitly describes serous retinal detachment as an
      ocular finding in severe VKH.
- category: Ophthalmologic
  name: Sunset Glow Fundus
  phenotype_term:
    preferred_term: Sunset glow fundus
    term:
      id: HP:0007894
      label: Fundus hypopigmentation
  evidence:
  - reference: DOI:10.1186/s12886-024-03511-9
    reference_title: Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      “Sunset glow fundus” was observed in 23.5% of the acute-resolved group and
      64.3% of the chronic-recurrent group patients (P = 0.001).
    explanation: >-
      This VKH cohort directly documents sunset glow fundus and its enrichment
      in chronic recurrent disease. Fundus hypopigmentation is used as the
      closest validated local HPO binding for the depigmented fundus finding.
- category: Neurologic
  name: Meningeal Irritation
  phenotype_term:
    preferred_term: Non-infectious meningitis
    term:
      id: HP:0033430
      label: Non-infectious meningitis
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease
      which targets melanin-containing tissues such as the uvea, meninges, ear
      and skin.
    explanation: >-
      Meningeal involvement supports the non-infectious meningitis or meningeal
      irritation component of VKH.
- category: Neurologic
  name: Headache
  phenotype_term:
    preferred_term: Headache
    term:
      id: HP:0002315
      label: Headache
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      Brain involvement in this multisystem inflammatory disease supports
      headache as a neurologic manifestation without relying on uncached
      full-text frequency data.
- category: Sensory
  name: Tinnitus
  phenotype_term:
    preferred_term: Tinnitus
    term:
      id: HP:0000360
      label: Tinnitus
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      Ear involvement directly supports auditory manifestations such as
      tinnitus without relying on uncached full-text frequency data.
- category: Sensory
  name: Sensorineural Hearing Loss
  phenotype_term:
    preferred_term: Sensorineural hearing impairment
    term:
      id: HP:0000407
      label: Sensorineural hearing impairment
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      The review supports auditory system involvement; the broader HPO hearing
      impairment term is used because the abstract does not distinguish tinnitus
      from hearing loss.
- category: Dermatologic
  name: Vitiligo
  phenotype_term:
    preferred_term: Vitiligo
    term:
      id: HP:0001045
      label: Vitiligo
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt-Koyanagi-Harada (VKH) disease is an idiopathic autoimmune disease
      which targets melanin-containing tissues such as the uvea, meninges, ear
      and skin.
    explanation: >-
      Skin involvement in a melanocyte-targeting disease supports vitiligo as an
      integumentary VKH phenotype.
- category: Dermatologic
  name: Poliosis
  phenotype_term:
    preferred_term: Poliosis
    term:
      id: HP:0002290
      label: Poliosis
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      Hair involvement in VKH supports poliosis as a hair depigmentation
      manifestation.
- category: Dermatologic
  name: Alopecia
  phenotype_term:
    preferred_term: Alopecia
    term:
      id: HP:0001596
      label: Alopecia
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      Hair involvement directly supports alopecia as an integumentary VKH
      manifestation without relying on uncached full-text frequency data.
genetic:
- name: HLA-DRB1*04:05 susceptibility
  gene_term:
    preferred_term: HLA-DRB1
    term:
      id: hgnc:4948
      label: HLA-DRB1
  presence: Susceptibility allele
  association: HLA-DRB1*04:05 is associated with susceptibility to VKH and VKH-like immune-related uveitis.
  relationship_type: RISK_FACTOR
  variant_origin: GERMLINE
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Different possible pathogenetic mechanisms underlying the onset of VKH
      after COVID-19 vaccination are discussed, while the presence of the HLA
      DR4 antigen as a genetic predisposition for the onset of the disease after
      COVID-19 infection and vaccination is proposed.
    explanation: >-
      The review supports HLA-DR4/HLA-DRB1-related genetic predisposition in
      triggered VKH contexts.
- name: PRKCD susceptibility locus
  gene_term:
    preferred_term: PRKCD
    term:
      id: hgnc:9399
      label: PRKCD
  presence: Susceptibility locus
  association: PRKCD rs74437127 alleles are associated with altered VKH susceptibility in a Chinese Han case-control cohort.
  relationship_type: SUSCEPTIBILITY
  variant_origin: GERMLINE
  evidence:
  - reference: DOI:10.1186/s40246-023-00459-7
    reference_title: Genetic association of PRKCD and CARD9 polymorphisms with Vogt-Koyanagi-Harada disease in the Chinese Han population
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      We found that rs74437127 C allele of PRKCD, rs3812555 CC genotype, and C
      allele of CARD9 were associated with increased susceptibility of VKH
      (Pc = 0.020, OR = 1.624; Pc = 2.04 × 10–5, OR = 1.810; Pc = 2.76 × 10–5,
      OR = 1.698, respectively).
    explanation: >-
      This human case-control study directly supports PRKCD as a susceptibility
      locus.
- name: CARD9 susceptibility locus
  gene_term:
    preferred_term: CARD9
    term:
      id: hgnc:16391
      label: CARD9
  presence: Susceptibility locus
  association: CARD9 rs3812555 is associated with VKH susceptibility and increased inflammatory cytokine production.
  relationship_type: SUSCEPTIBILITY
  variant_origin: GERMLINE
  evidence:
  - reference: DOI:10.1186/s40246-023-00459-7
    reference_title: Genetic association of PRKCD and CARD9 polymorphisms with Vogt-Koyanagi-Harada disease in the Chinese Han population
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      We found an association between PRKCD rs74437127 and CARD9 rs3812555
      polymorphisms and VKH susceptibility and revealed that the increased
      susceptibility of rs3812555 for VKH may be mediated by regulating CARD9
      gene expression and the production of pro-inflammatory cytokines, such as
      TNF-α.
    explanation: >-
      This conclusion supports CARD9 as a susceptibility locus with a plausible
      cytokine-mediated functional correlate.
environmental:
- name: SARS-CoV-2 infection or vaccination
  presence: Reported trigger
  description: >-
    COVID-19 infection and SARS-CoV-2 vaccination have been reported before VKH
    onset or relapse. The association is treated as a trigger context rather
    than proof of a necessary cause.
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      The Coronavirus Disease 2019 (COVID-19) is a new contagious infection that
      might trigger the onset of VKH disease, as previously proposed for other
      viruses.
    explanation: >-
      The review supports SARS-CoV-2 infection as a reported possible trigger.
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Moreover, after the mass vaccination against SARS-CoV-2 worldwide, cases
      of VKH disease associated with COVID-19 vaccination have been reported.
    explanation: >-
      The review supports COVID-19 vaccination as a reported onset or relapse
      context.
- name: Immune checkpoint inhibitor therapy
  presence: Reported iatrogenic trigger
  description: >-
    Immune checkpoint inhibitor therapy can be associated with VKH-like
    immune-related uveitis, especially in HLA-DRB1*04:05-positive patients.
  evidence:
  - reference: DOI:10.1038/s41598-023-40565-z
    reference_title: "HLA-DRB1*04:05 is involved in the development of Vogt–Koyanagi–Harada disease-like immune-related adverse events in patients receiving immune checkpoint inhibitors"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Statistical analysis showed that HLA-DRB1*04:05 was significantly
      associated with developing VKH-like ICIU (P = 0.029).
    explanation: >-
      This case-series abstract supports the reported HLA-DRB1*04:05-associated
      VKH-like immune checkpoint inhibitor uveitis context.
progression:
- phase: Prodromal neurologic and auditory phase
  duration: days
  notes: >-
    The prodromal phase is represented by extraocular neurologic and auditory
    symptoms before or around ocular disease onset.
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      Ear and brain involvement supports the prodromal extraocular symptom
      context described in the Falcon report.
- phase: Acute uveitic phase
  duration: weeks
  notes: >-
    Acute VKH centers on bilateral uveitic and choroidal inflammation with
    subretinal fluid and possible optic nerve involvement.
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      This typically presents in the eye with acute findings of granulomatous
      anterior uveitis, diffuse choroidal thickening, multiple focal areas of
      sub-retinal fluid and, in severe cases, optic nerve involvement with
      bullous serous retinal detachment can occur.
    explanation: >-
      This directly supports the acute uveitic phase.
- phase: Convalescent depigmentation phase
  notes: >-
    The convalescent phase includes depigmenting signs in melanocyte-containing
    tissues, including skin and hair manifestations.
  evidence:
  - reference: DOI:10.3390/jcm12196242
    reference_title: Vogt-Koyanagi-Harada Disease and COVID
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Vogt–Koyanagi–Harada (VKH) is a rare multisystem inflammatory disease
      affecting the eyes, ears, brain, skin, and hair.
    explanation: >-
      Skin and hair involvement supports the convalescent integumentary
      manifestations described in the Falcon report.
- phase: Chronic recurrent phase
  notes: >-
    VKH may resolve after acute treatment or progress to a chronic recurrent
    phase. Older age at onset and sunset glow fundus are reported predictors of
    chronic recurrence.
  evidence:
  - reference: DOI:10.1186/s12886-024-03511-9
    reference_title: Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Poor initial BCVA (P = 0.046) and the occurrence of “sunset glow fundus”
      (P = 0.040) were significantly associated with progression to the chronic
      recurrent phase.
    explanation: >-
      This retrospective cohort identifies clinical predictors of chronic
      recurrent VKH.
diagnosis:
- name: Multimodal Ocular Imaging and Clinical Criteria
  description: >-
    VKH diagnosis is clinical and supported by multimodal ocular imaging,
    including OCT/EDI-OCT, fluorescein angiography, indocyanine green
    angiography, and OCT angiography in appropriate contexts.
  diagnosis_term:
    preferred_term: diagnostic procedure
    term:
      id: MAXO:0000003
      label: diagnostic procedure
  results: >-
    Imaging documents choroidal thickening, subretinal fluid, and inflammatory
    activity that supports VKH diagnosis and monitoring.
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      diffuse choroidal thickening, multiple focal areas of sub-retinal fluid
      and, in severe cases, optic nerve involvement with bullous serous retinal
      detachment can occur.
    explanation: >-
      These ocular findings are the target abnormalities evaluated by VKH
      diagnostic imaging.
treatments:
- name: Early Systemic Corticosteroids
  description: >-
    High-dose systemic corticosteroids are used early to suppress acute ocular
    and systemic inflammation, commonly followed by early immunomodulatory
    therapy to reduce chronic progression risk.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: corticosteroid
      term:
        id: CHEBI:50858
        label: corticosteroid
  target_phenotypes:
  - preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Treatment is usually initiated with corticosteroids followed by an early
      introduction of immunosuppressive treatment (IMT) to achieve immediate
      response after disease presentation, although the choice of IMT for VKH
      can vary.
    explanation: >-
      This clinical review supports systemic corticosteroids as the initial VKH
      treatment backbone.
- name: Combined Immunomodulatory Therapy
  description: >-
    Conventional steroid-sparing immunomodulatory therapy, including agents such
    as mycophenolate mofetil or cyclosporine, is used with low-dose steroids to
    stabilize disease and preserve vision.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: mycophenolate mofetil
      term:
        id: CHEBI:8764
        label: mycophenolate mofetil
    - preferred_term: cyclosporin A
      term:
        id: CHEBI:4031
        label: cyclosporin A
  target_phenotypes:
  - preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: DOI:10.1186/s12348-023-00333-6
    reference_title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      81% (21 of 26 patients) of our patients treated with combined IMT/steroid
      were able to achieve disease stability with significant good visual
      outcome at 24 months (Median VApre-IMT = 0.3 Logmar vs VApost-IMT = 0.0
      Logmar, p = 0.0001).
    explanation: >-
      This retrospective clinical series supports combined immunomodulatory
      therapy and low-dose steroid treatment for VKH disease stability.
  - reference: DOI:10.1038/s41467-023-39483-5
    reference_title: A randomized non-inferiority trial of therapeutic strategy with immunosuppressants versus biologics for Vogt-Koyanagi-Harada disease
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Here, we report that combined with a non-standard corticosteroid regimen,
      cyclosporine-based immunosuppressant strategy is non-inferior to
      adalimumab-based biologic strategy by 26 weeks for visual improvement in a
      cohort of patients with Vogt-Koyanagi-Harada disease, 75% of whom have a
      late-phase disease.
    explanation: >-
      The randomized non-inferiority trial supports cyclosporine-based
      conventional immunosuppression as an evidence-backed therapeutic strategy.
- name: Adalimumab Biologic Therapy
  description: >-
    Adalimumab, an anti-TNF monoclonal antibody, is used for refractory or
    chronic recurrent VKH and is under study for acute VKH in combination with
    glucocorticoids.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: adalimumab
      term:
        id: NCIT:C65216
        label: Adalimumab
  target_phenotypes:
  - preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  target_mechanisms:
  - target: Cytokine-Amplified Chronic Recurrent Inflammation
    treatment_effect: INHIBITS
    description: >-
      Anti-TNF biologic therapy is represented as reducing cytokine-amplified
      chronic recurrent ocular inflammation.
  evidence:
  - reference: DOI:10.1186/s12886-024-03511-9
    reference_title: Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      ADA significantly reduced anterior chamber inflammatory cells (P = 0.000)
      and vitreous cavity inflammatory cells (P = 0.001) in the chronic-recurrent
      group, and markedly decreased the recurrence rate in VKH patients (P = 0.009).
    explanation: >-
      This retrospective cohort directly supports adalimumab efficacy in chronic
      recurrent VKH.
- name: Janus Kinase Inhibitor Therapy
  description: >-
    JAK inhibitors are an emerging option for refractory non-infectious ocular
    inflammatory disease; available evidence includes a prospective registry
    cohort with one VKH patient, so this is promising but not VKH-specific
    definitive evidence.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: baricitinib
      term:
        id: CHEBI:95341
        label: baricitinib
    - preferred_term: tofacitinib
      term:
        id: CHEBI:71200
        label: tofacitinib
  target_phenotypes:
  - preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: DOI:10.3389/fmed.2024.1439338
    reference_title: "Efficacy and safety of Janus kinase inhibitors in non-infectious inflammatory ocular diseases: a prospective cohort study from the international AIDA network registries"
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Ocular inflammation was related to a systemic disease in 8 (66.7%)
      patients as follows: spondyloarthritis (n = 3), peripheral psoriatic
      arthritis (n = 1), rheumatoid arthritis (n = 1), antinuclear antibodies
      (ANA) positive juvenile idiopathic arthritis (n = 1), Behçet’s syndrome
      (n = 1), Vogt-Koyanagi-Harada syndrome (n = 1).
    explanation: >-
      The prospective registry includes a VKH case among non-infectious ocular
      inflammatory diseases, so it partially supports JAK inhibitor relevance
      without establishing VKH-specific efficacy.
clinical_trials:
- name: NCT03399175
  phase: NOT_APPLICABLE
  status: RECRUITING
  description: >-
    Prospective study of early systemic high-dose corticosteroid and
    immunosuppressive therapy from VKH disease onset with multimodal clinical
    follow-up.
  target_phenotypes:
  - preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: clinicaltrials:NCT03399175
    reference_title: "Influência de imunomodulação Precoce Influence of Early Immunosuppressive Therapy on the Course of Vogt-Koyanagi-Harada Disease: a Prospective Study"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      This prospective study will include patients with Vogt-Koyanagi-Harada
      disease from disease onset, treated with early systemic high-dose
      corticosteroid and immunosuppressive therapy.
    explanation: >-
      ClinicalTrials.gov identifies this VKH study of early systemic
      corticosteroid plus immunosuppressive therapy.
- name: NCT05590416
  phase: NOT_APPLICABLE
  status: RECRUITING
  description: >-
    Observational study of adalimumab treatment in acute Vogt-Koyanagi-Harada
    disease.
  target_phenotypes:
  - preferred_term: Panuveitis
    term:
      id: HP:0012121
      label: Panuveitis
  evidence:
  - reference: clinicaltrials:NCT05590416
    reference_title: An Observational Study of Adalimumab in the Treatment of Acute Vogt-Koyanagi-Harada Disease
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      This project is designed to test the hypothesis that adalimumab is
      clinically useful for patients with acuta Vogt-Koyanagi-Harada disease
    explanation: >-
      ClinicalTrials.gov identifies an acute VKH observational study of
      adalimumab.
references:
- reference: DOI:10.3390/jcm12196242
  title: Vogt-Koyanagi-Harada Disease and COVID
  findings: []
- reference: DOI:10.1186/s12348-023-00333-6
  title: "Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature"
  findings: []
- reference: DOI:10.1186/s40246-023-00459-7
  title: Genetic association of PRKCD and CARD9 polymorphisms with Vogt-Koyanagi-Harada disease in the Chinese Han population
  findings: []
- reference: DOI:10.1038/s41598-023-40565-z
  title: "HLA-DRB1*04:05 is involved in the development of Vogt-Koyanagi-Harada disease-like immune-related adverse events in patients receiving immune checkpoint inhibitors"
  findings: []
- reference: DOI:10.1038/s41467-023-39483-5
  title: A randomized non-inferiority trial of therapeutic strategy with immunosuppressants versus biologics for Vogt-Koyanagi-Harada disease
  findings: []
- reference: DOI:10.1186/s12886-024-03511-9
  title: Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease
  findings: []
- reference: DOI:10.3389/fmed.2024.1439338
  title: "Efficacy and safety of Janus kinase inhibitors in non-infectious inflammatory ocular diseases: a prospective cohort study from the international AIDA network registries"
  findings: []
- reference: clinicaltrials:NCT03399175
  title: "Influência de imunomodulação Precoce Influence of Early Immunosuppressive Therapy on the Course of Vogt-Koyanagi-Harada Disease: a Prospective Study"
  findings: []
- reference: clinicaltrials:NCT05590416
  title: An Observational Study of Adalimumab in the Treatment of Acute Vogt-Koyanagi-Harada Disease
  findings: []
📚

References & Deep Research

References

9
DOI:10.3390/jcm12196242
Vogt-Koyanagi-Harada Disease and COVID
No top-level findings curated for this source.
DOI:10.1186/s12348-023-00333-6
Immunosuppressive therapy for Vogt-Koyanagi-Harada disease: a retrospective study and review of literature
No top-level findings curated for this source.
DOI:10.1186/s40246-023-00459-7
Genetic association of PRKCD and CARD9 polymorphisms with Vogt-Koyanagi-Harada disease in the Chinese Han population
No top-level findings curated for this source.
DOI:10.1038/s41598-023-40565-z
HLA-DRB1*04:05 is involved in the development of Vogt-Koyanagi-Harada disease-like immune-related adverse events in patients receiving immune checkpoint inhibitors
No top-level findings curated for this source.
DOI:10.1038/s41467-023-39483-5
A randomized non-inferiority trial of therapeutic strategy with immunosuppressants versus biologics for Vogt-Koyanagi-Harada disease
No top-level findings curated for this source.
DOI:10.1186/s12886-024-03511-9
Predictive factors and adalimumab efficacy in managing chronic recurrence Vogt-Koyanagi-Harada disease
No top-level findings curated for this source.
DOI:10.3389/fmed.2024.1439338
Efficacy and safety of Janus kinase inhibitors in non-infectious inflammatory ocular diseases: a prospective cohort study from the international AIDA network registries
No top-level findings curated for this source.
clinicaltrials:NCT03399175
Influência de imunomodulação Precoce Influence of Early Immunosuppressive Therapy on the Course of Vogt-Koyanagi-Harada Disease: a Prospective Study
No top-level findings curated for this source.
clinicaltrials:NCT05590416
An Observational Study of Adalimumab in the Treatment of Acute Vogt-Koyanagi-Harada Disease
No top-level findings curated for this source.

Deep Research

1
Falcon ▸
Disease Characteristics Research Template
Edison Scientific Literature 47 citations 2026-05-11T09:24:35.162671

Question: You are an expert researcher providing comprehensive, well-cited information.

Provide detailed information focusing on: 1. Key concepts and definitions with current understanding 2. Recent developments and latest research (prioritize 2023-2024 sources) 3. Current applications and real-world implementations 4. Expert opinions and analysis from authoritative sources 5. Relevant statistics and data from recent studies

Format as a comprehensive research report with proper citations. Include URLs and publication dates where available. Always prioritize recent, authoritative sources and provide specific citations for all major claims.

Disease Characteristics Research Template

Target Disease

  • Disease Name: Vogt-Koyanagi-Harada Disease
  • MONDO ID: (if available)
  • Category: Autoimmune

Research Objectives

Please provide a comprehensive research report on Vogt-Koyanagi-Harada Disease covering all of the disease characteristics listed below. This report will be used to populate a disease knowledge base entry. Be thorough and cite primary literature (PMID preferred) for all claims.

For each section, suggested databases/resources are listed. These are the first places you should search for information on each topic.

1. Disease Information

Search first: OMIM, Orphanet, ICD-10/ICD-11, MeSH, PubMed

  • What is the disease? Provide a concise overview.
  • What are the key identifiers? (OMIM, Orphanet, ICD-10/ICD-11, MeSH, Mondo)
  • What are the common synonyms and alternative names?
  • Is the information derived from individual patients (e.g., EHR) or aggregated disease-level resources?

2. Etiology

  • Disease Causal Factors: What are the primary causes? (genetic, environmental, infectious, mechanistic)
  • Risk Factors:

    Search first: PubMed, Cochrane Library, UpToDate, clinical guidelines, ClinVar, ClinGen, GWAS Catalog, PheGenI, CTD, CDC, WHO, epidemiological databases

  • Genetic risk factors (causal variants, susceptibility loci, modifier genes)
  • Environmental risk factors (toxins, lifestyle, occupational exposures, age, sex, family history)
  • Protective Factors:

    Search first: PubMed, Cochrane Library, clinical trial databases, GWAS Catalog, gnomAD, WHO, CDC, nutrition databases

  • Genetic protective factors (protective variants, modifier alleles)
  • Environmental protective factors (diet, lifestyle, exposures that reduce risk)
  • Gene-Environment Interactions: How do genetic and environmental factors interact to influence disease?

    Search first: CTD, PubMed, PheGenI, GxE databases

3. Phenotypes

Search first: HPO (Human Phenotype Ontology), OMIM, Orphanet, PubMed, clinicaltrials.gov, MedDRA, SNOMED CT, DECIPHER, LOINC

For each phenotype, provide: - Phenotype type: symptoms, clinical signs, physical manifestations, behavioral changes, or laboratory abnormalities

For symptoms/signs: HPO, OMIM, Orphanet, PubMed For behavioral changes: HPO, DSM, RDoC (Research Domain Criteria), PubMed For laboratory abnormalities: LOINC, SNOMED CT, LabTests Online, PubMed - Phenotype characteristics: Search first: OMIM, Orphanet, HPO, PubMed - Age of symptom onset (neonatal, childhood, adult-onset, late-onset) - Symptom severity (mild, moderate, severe, variable) - Symptom progression (stable, progressive, episodic, fluctuating) - Frequency among affected individuals (percentage or qualitative) - Quality of life impact: Effects on daily functioning and well-being (per-phenotype when possible) Search first: EQ-5D database, SF-36, WHO QOL databases, PubMed - Suggest HPO (Human Phenotype Ontology) terms for each phenotype

4. Genetic/Molecular Information

  • Causal Genes: Gene mutations or chromosomal abnormalities responsible for disease (gene symbols, OMIM IDs)

    Search first: OMIM, ClinVar, HGMD, Ensembl, NCBI Gene

  • Pathogenic Variants:
  • Affected genes (gene symbols, HGNC IDs) > Search first: OMIM, NCBI Gene, Ensembl, HGNC, UniProt, GeneCards
  • Variant classification (pathogenic, likely pathogenic, VUS per ACMG/AMP guidelines) > Search first: ClinVar, ClinGen, ACMG/AMP guidelines, VarSome
  • Variant type/class (missense, frameshift, nonsense, splice-site, structural)
  • Allele frequency in population databases > Search first: gnomAD, 1000 Genomes, ExAC, TOPMed, dbSNP
  • Somatic vs germline origin > Search first: COSMIC (somatic), ClinVar, ICGC, TCGA
  • Functional consequences (loss of function, gain of function, dominant negative)
  • Modifier Genes: Genes that modify disease severity or expression
  • Epigenetic Information: DNA methylation, histone modifications, chromatin changes affecting disease

    Search first: ENCODE, Roadmap Epigenomics, MethBase, DiseaseMeth

  • Chromosomal Abnormalities: Large-scale genetic changes (aneuploidy, translocations, inversions)

    Search first: DECIPHER, ClinVar, ECARUCA, UCSC Genome Browser

5. Environmental Information

  • Environmental Factors: Non-genetic contributing factors (toxins, radiation, pollution, occupational exposure)

    Search first: CTD (Comparative Toxicogenomics Database), TOXNET, PubMed, EPA databases

  • Lifestyle Factors: Behavioral factors (smoking, diet, exercise, alcohol consumption)

    Search first: CDC databases, WHO, PubMed, NHANES

  • Infectious Agents: If applicable, pathogens causing or triggering disease (bacteria, viruses, fungi, parasites)

    Search first: NCBI Taxonomy, ViPR, BV-BRC, MicrobeDB, GIDEON

6. Mechanism / Pathophysiology

  • Molecular Pathways: Specific signaling cascades or biochemical pathways involved (Wnt, MAPK, mTOR, PI3K-AKT, etc.)

    Search first: KEGG, Reactome, WikiPathways, PathBank, BioCyc

  • Cellular Processes: Cell-level mechanisms (apoptosis, autophagy, cell cycle dysregulation, inflammation, etc.)

    Search first: Gene Ontology (GO), Reactome, KEGG, PubMed

  • Protein Dysfunction: How protein structure or function is altered (misfolding, aggregation, loss of function, gain of function)

    Search first: UniProt, PDB (Protein Data Bank), InterPro, Pfam, AlphaFold

  • Metabolic Changes: Alterations in metabolic processes (energy metabolism, lipid metabolism, amino acid metabolism)

    Search first: KEGG, BioCyc, HMDB (Human Metabolome Database), BRENDA

  • Immune System Involvement: Role of immune response (autoimmunity, immunodeficiency, chronic inflammation)

    Search first: ImmPort, Immunome Database, IEDB, Gene Ontology

  • Tissue Damage Mechanisms: How tissues/ are injured (oxidative stress, ischemia, fibrosis, necrosis)

    Search first: PubMed, Gene Ontology, Reactome

  • Biochemical Abnormalities: Specific molecular defects (enzyme deficiencies, receptor dysfunction, ion channel defects)

    Search first: BRENDA, UniProt, KEGG, OMIM, PubMed

  • Epigenetic Changes: DNA methylation, histone modifications affecting gene expression in disease

    Search first: ENCODE, Roadmap Epigenomics, MethBase, DiseaseMeth

  • Molecular Profiling (if available):
  • Transcriptomics/gene expression changes > Search first: GEO (Gene Expression Omnibus), ArrayExpress, GTEx, Human Cell Atlas, SRA
  • Proteomics findings > Search first: PRIDE, ProteomeXchange, Human Protein Atlas, STRING, BioGRID
  • Metabolomics signatures > Search first: MetaboLights, Metabolomics Workbench, HMDB, METLIN
  • Lipidomics alterations > Search first: LIPID MAPS, SwissLipids, LipidHome, Metabolomics Workbench
  • Genomic structural features > Search first: UCSC Genome Browser, Ensembl, NCBI, dbVar, DGV
  • Advanced Technologies (if applicable):
  • Single-cell analysis findings (cell-type specific mechanisms, cellular heterogeneity) > Search first: Human Cell Atlas, Single Cell Portal, GEO, CELLxGENE
  • Spatial transcriptomics findings > Search first: GEO, Spatial Research, Vizgen, 10x Genomics data
  • Multi-omics integration results > Search first: TCGA, ICGC, cBioPortal, LinkedOmics, PubMed
  • Functional genomics screens (CRISPR, RNAi) > Search first: DepMap, GenomeRNAi, PubMed, BioGRID ORCS

For each mechanism, describe: - The causal chain from initial trigger to clinical manifestation - Which mechanisms are upstream vs downstream - What cell types and biological processes are involved - Suggest GO terms for biological processes and CL terms for cell types

7. Anatomical Structures Affected

  • Organ Level:
  • Primary organs directly affected
  • Secondary organ involvement (complications, secondary effects)
  • Body systems involved (cardiovascular, nervous, digestive, respiratory, endocrine, etc.)

    Search first: Uberon, FMA (Foundational Model of Anatomy), OMIM, HPO, ICD-11, MeSH, SNOMED CT

  • Tissue and Cell Level:
  • Specific tissue types affected (epithelial, connective, muscle, nervous)
  • Specific cell populations targeted (with Cell Ontology terms)

    Search first: Uberon, Human Protein Atlas, Cell Ontology, Human Cell Atlas, CellMarker, PanglaoDB

  • Subcellular Level:
  • Cellular compartments involved (mitochondria, nucleus, ER, lysosomes) (with GO Cellular Component terms)

    Search first: Gene Ontology (Cellular Component), UniProt, Human Protein Atlas

  • Localization:
  • Specific anatomical sites (with UBERON terms) > Search first: FMA, Uberon, NeuroNames (for brain), SNOMED CT
  • Lateralization (unilateral, bilateral, asymmetric) > Search first: HPO, clinical literature, imaging databases

8. Temporal Development

  • Onset:
  • Typical age of onset (congenital, pediatric, adult, geriatric)
  • Onset pattern (acute, subacute, chronic, insidious)

    Search first: OMIM, Orphanet, HPO, PubMed

  • Progression:
  • Disease stages (early, intermediate, advanced, end-stage) > Search first: Cancer Staging Manual (AJCC), WHO classifications, PubMed
  • Progression rate (rapid, slow, variable)
  • Disease course pattern (episodic, relapsing-remitting, progressive, stable)
  • Disease duration (self-limited, chronic lifelong)

    Search first: Disease registries, longitudinal cohort databases, natural history studies, PubMed, Orphanet, OMIM

  • Patterns:
  • Remission patterns (spontaneous, treatment-induced) > Search first: Clinical trial databases, disease registries, PubMed
  • Critical periods (time windows of vulnerability or opportunity for intervention) > Search first: PubMed, developmental biology databases, clinical guidelines

9. Inheritance and Population

  • Epidemiology:
  • Prevalence (cases per 100,000 at given time)
  • Incidence (new cases per 100,000 per year)

    Search first: Orphanet, CDC, WHO, GBD (Global Burden of Disease), national registries, SEER, disease registries

  • For Genetic Etiology:
  • Inheritance pattern (AD, AR, X-linked, mitochondrial, multifactorial, polygenic) > Search first: OMIM, Orphanet, ClinVar, GTR (Genetic Testing Registry)
  • Penetrance (complete, incomplete, age-dependent) > Search first: ClinVar, OMIM, PubMed, ClinGen
  • Expressivity (variable, consistent) > Search first: OMIM, ClinVar, PubMed
  • Genetic anticipation (increasing severity in successive generations) > Search first: OMIM, PubMed (especially for repeat expansion disorders)
  • Germline mosaicism > Search first: ClinVar, OMIM, genetic counseling literature, PubMed
  • Founder effects (population-specific mutations) > Search first: gnomAD, population genetics databases, PubMed
  • Consanguinity role > Search first: OMIM, population studies, genetic counseling resources
  • Carrier frequency > Search first: gnomAD, carrier screening databases, GeneReviews, GTR
  • Population Demographics:
  • Affected populations (ethnic or demographic groups with higher prevalence) > Search first: gnomAD, 1000 Genomes, PAGE Study, PubMed, population registries
  • Geographic distribution (endemic areas, regional variation) > Search first: WHO, CDC, GBD, Orphanet, geographic epidemiology databases
  • Geographic distribution of specific variants
  • Sex ratio (male:female) > Search first: Disease registries, OMIM, PubMed, epidemiological databases
  • Age distribution of affected individuals > Search first: CDC, disease registries, SEER, Orphanet

10. Diagnostics

  • Clinical Tests:
  • Laboratory tests (blood, urine, tissue chemistry, specific enzyme assays) > Search first: LOINC, LabTests Online, PubMed
  • Biomarkers (proteins, metabolites, genetic markers, circulating biomarkers) > Search first: FDA Biomarker List, BEST (Biomarkers, EndpointS, and other Tools), PubMed
  • Imaging studies (X-ray, CT, MRI, PET, ultrasound) > Search first: RadLex, DICOM, Radiopaedia, imaging databases
  • Functional tests (pulmonary function, cardiac stress tests) > Search first: LOINC, clinical guidelines, PubMed
  • Electrophysiology (EEG, EMG, ECG, nerve conduction studies) > Search first: LOINC, clinical neurophysiology databases, PubMed
  • Biopsy findings (histopathology, immunohistochemistry) > Search first: SNOMED CT, College of American Pathologists resources, PubMed
  • Pathology findings (microscopic examination) > Search first: SNOMED CT, Digital Pathology databases, PubMed
  • Genetic Testing:

    Search first: GTR (Genetic Testing Registry), GeneReviews, ClinGen

  • Overview of recommended genetic testing approach
  • Whole genome sequencing (WGS) utility > Search first: GTR, ClinVar, GEL (Genomics England), gnomAD
  • Whole exome sequencing (WES) utility > Search first: GTR, ClinVar, OMIM, GeneMatcher
  • Gene panels (which panels, which genes) > Search first: GTR, ClinVar, laboratory-specific databases
  • Single gene testing > Search first: GTR, ClinVar, OMIM, GeneReviews
  • Chromosomal microarray (CMA) > Search first: DECIPHER, ClinVar, dbVar, ECARUCA
  • Karyotyping > Search first: Chromosome Abnormality Database, ClinVar, cytogenetics resources
  • FISH > Search first: ClinVar, cytogenetics databases, PubMed
  • Mitochondrial DNA testing > Search first: MITOMAP, MSeqDR, ClinVar, GTR
  • Repeat expansion testing > Search first: GTR, ClinVar, repeat expansion databases, PubMed
  • Omics-Based Diagnostics (if applicable):
  • RNA sequencing / transcriptomics > Search first: GEO, ArrayExpress, GTEx, RNA-seq databases
  • Proteomics > Search first: PRIDE, ProteomeXchange, FDA Biomarker database
  • Metabolomics > Search first: MetaboLights, Metabolomics Workbench, HMDB
  • Epigenomics > Search first: GEO, ENCODE, Roadmap Epigenomics, MethBase
  • Liquid biopsy > Search first: COSMIC, ClinVar, liquid biopsy databases, PubMed
  • Clinical Criteria:
  • Standardized diagnostic criteria (DSM, ICD, society guidelines) > Search first: DSM-5, ICD-11, clinical society guidelines, UpToDate
  • Differential diagnosis (other conditions to rule out, with distinguishing features) > Search first: DynaMed, UpToDate, clinical decision support systems
  • Screening:
  • Screening methods for asymptomatic individuals (newborn screening, carrier screening, cascade screening) > Search first: ACMG recommendations, CDC newborn screening, GTR

11. Outcome/Prognosis

  • Survival and Mortality:
  • Survival rate (5-year, 10-year, overall) > Search first: SEER, cancer registries, disease-specific registries, PubMed
  • Life expectancy (with and without treatment if applicable) > Search first: Orphanet, disease registries, actuarial databases, PubMed
  • Mortality rate > Search first: CDC, WHO, GBD, national mortality databases
  • Disease-specific mortality (deaths directly attributable to disease) > Search first: Disease registries, CDC Wonder, GBD, PubMed
  • Morbidity and Function:
  • Morbidity (disease-related disability and health impacts) > Search first: GBD, WHO, disability databases, PubMed
  • Disability outcomes (long-term functional impairments) > Search first: ICF (International Classification of Functioning), disability registries
  • Quality of life measures (EQ-5D, SF-36, PROMIS, disease-specific tools) > Search first: EQ-5D database, SF-36, PROMIS, PubMed
  • Disease Course:
  • Complications (secondary problems: infections, organ failure, etc.) > Search first: ICD codes, disease registries, clinical databases, PubMed
  • Recovery potential (likelihood and extent of recovery, with vs without treatment) > Search first: Natural history studies, rehabilitation databases, PubMed
  • Prediction:
  • Prognostic factors (age, disease severity, biomarkers, treatment response) > Search first: Prognostic models databases, clinical calculators, PubMed
  • Prognostic biomarkers (molecular markers predicting disease course) > Search first: FDA Biomarker database, PubMed, cancer prognostic databases

12. Treatment

  • Pharmacotherapy:
  • Pharmacological treatments (drug names, drug classes, mechanisms of action) > Search first: DrugBank, RxNorm, ATC classification, DailyMed, FDA databases
  • Pharmacogenomics (how genetic variants affect drug metabolism, efficacy, toxicity) > Search first: PharmGKB, CPIC (Clinical Pharmacogenetics), FDA Table of PGx Biomarkers
  • Advanced Therapeutics:
  • Gene therapy (viral vectors, CRISPR, gene replacement, gene editing) > Search first: ClinicalTrials.gov, FDA gene therapy database, ASGCT resources
  • Cell therapy (stem cell transplant, CAR-T, cellular therapeutics) > Search first: ClinicalTrials.gov, FDA cell therapy database, FACT standards
  • RNA-based therapies (ASOs, siRNA, mRNA therapies) > Search first: ClinicalTrials.gov, FDA approvals, PubMed
  • Targeted therapies (treatments directed at specific molecular targets) > Search first: My Cancer Genome, OncoKB, ClinicalTrials.gov, FDA approvals
  • Immunotherapies (checkpoint inhibitors, monoclonal antibodies) > Search first: Cancer Immunotherapy Database, FDA approvals, ClinicalTrials.gov
  • Surgical and Interventional:
  • Surgical interventions (types of surgery, timing, outcomes) > Search first: CPT codes, surgical registries, clinical guidelines, PubMed
  • Supportive and Rehabilitative:
  • Supportive care (symptom management, pain control, nutrition) > Search first: Clinical guidelines, Cochrane Library, PubMed
  • Rehabilitation (physical therapy, occupational therapy, speech therapy) > Search first: Rehabilitation medicine databases, clinical guidelines, PubMed
  • Experimental:
  • Experimental treatments in clinical trials (with NCT identifiers if available) > Search first: ClinicalTrials.gov, EU Clinical Trials Register, WHO ICTRP
  • Treatment Outcomes:
  • Treatment response rates > Search first: Clinical trial databases, FDA reviews, systematic reviews, PubMed
  • Side effects and adverse events > Search first: FDA Adverse Event Reporting System (FAERS), MedWatch, PubMed
  • Treatment Strategy:
  • Treatment algorithms (clinical pathways, decision trees) > Search first: Clinical practice guidelines, NCCN Guidelines, UpToDate
  • Combination therapies > Search first: ClinicalTrials.gov, treatment guidelines, PubMed
  • Personalized medicine approaches (genotype-guided treatment) > Search first: My Cancer Genome, CIViC, PharmGKB, precision medicine databases

For each treatment, suggest MAXO (Medical Action Ontology) terms where applicable.

13. Prevention

  • Prevention Levels:
  • Primary prevention (preventing disease occurrence: vaccination, risk factor modification) > Search first: CDC, WHO, USPSTF recommendations, Cochrane Library
  • Secondary prevention (early detection and treatment: screening programs, early intervention) > Search first: USPSTF, CDC screening guidelines, WHO
  • Tertiary prevention (preventing complications in those with disease) > Search first: Clinical guidelines, disease management protocols, PubMed
  • Immunization: Vaccine strategies (if applicable)

    Search first: CDC vaccine schedules, WHO immunization, FDA vaccine database

  • Screening and Early Detection:
  • Screening programs (population-based: newborn screening, cancer screening) > Search first: CDC screening programs, USPSTF, cancer screening databases
  • Genetic screening (carrier screening, preimplantation genetic diagnosis, prenatal testing) > Search first: ACMG recommendations, ACOG guidelines, GTR
  • Risk stratification (identifying high-risk individuals for targeted prevention) > Search first: Risk prediction models, clinical calculators, PubMed
  • Behavioral Interventions: Lifestyle modifications to reduce risk

    Search first: CDC, WHO, behavioral intervention databases, Cochrane Library

  • Counseling: Genetic counseling (risk assessment, family planning guidance)

    Search first: NSGC resources, ACMG guidelines, GeneReviews

  • Public Health:
  • Public health interventions (sanitation, vector control, health education) > Search first: CDC, WHO, public health databases, PubMed
  • Environmental interventions (reducing environmental risk factors) > Search first: EPA databases, WHO environmental health, PubMed
  • Prophylaxis: Preventive medications or procedures

    Search first: Clinical guidelines, FDA approvals, PubMed

14. Other Species / Natural Disease

  • Taxonomy: Species affected (with NCBI Taxon identifiers)

    Search first: NCBI Taxonomy

  • Breed: Specific breeds affected (with VBO identifiers if applicable)

    Search first: VBO (Vertebrate Breed Ontology)

  • Gene: Orthologous genes in other species (with NCBI Gene IDs)

    Search first: NCBI Gene

  • Natural Disease:
  • Naturally occurring disease in other species (companion animals, wildlife) > Search first: OMIA (Online Mendelian Inheritance in Animals), VetCompass, PubMed
  • Veterinary relevance and importance in animal health > Search first: OMIA, veterinary databases, PubMed
  • Comparative Biology:
  • Comparative pathology (similarities and differences across species) > Search first: OMIA, comparative pathology databases, PubMed
  • Evolutionary conservation of disease mechanisms > Search first: HomoloGene, OrthoMCL, Alliance of Genome Resources
  • Transmission (if applicable):
  • Zoonotic potential > Search first: CDC zoonotic diseases, WHO zoonoses, GIDEON
  • Cross-species susceptibility > Search first: NCBI Taxonomy, veterinary databases, PubMed

15. Model Organisms

  • Model Types:
  • Model organism type (mammalian, invertebrate, cellular, in vitro) > Search first: Alliance of Genome Resources, model organism databases
  • Specific model systems (mouse, rat, zebrafish, Drosophila, C. elegans, yeast, cell lines, organoids, iPSCs) > Search first: MGI, RGD, ZFIN, FlyBase, WormBase, SGD, ATCC, Cellosaurus
  • Induced models (drug treatment, surgical intervention, environmental manipulation) > Search first: MGI, model organism databases, PubMed
  • Genetic Models:
  • Types available (knockout, knock-in, transgenic, conditional, humanized) > Search first: MGI, IMPC, KOMP, EuMMCR, IMSR
  • Model Characteristics:
  • Phenotype recapitulation (how well model reproduces human disease features) > Search first: Model organism databases, comparative studies, PubMed
  • Model limitations (aspects of human disease not captured) > Search first: Model organism databases, PubMed, review articles
  • Applications:
  • Research applications (what aspects of disease can be studied) > Search first: Model organism databases, PubMed
  • Resources:
  • Model databases > Search first: MGI, RGD, ZFIN, FlyBase, WormBase, IMSR, EMMA, MMRRC

Citation Requirements

  • Cite primary literature (PMID preferred) for all mechanistic and clinical claims
  • Prioritize recent reviews and landmark papers
  • Include direct quotes from abstracts where possible to support key statements
  • Distinguish evidence source types: human clinical, model organism, in vitro, computational

Output Format

Structure your response as a comprehensive narrative organized by the sections above. For each section, provide: - Factual content with specific details (numbers, percentages, gene names, variant nomenclature) - Ontology term suggestions (HPO, GO, CL, UBERON, CHEBI, MAXO, MONDO) where applicable - Evidence citations with PMIDs - Direct quotes from abstracts to support key claims - Clear indication when information is not available or not applicable for this disease

This report will be used to populate a disease knowledge base entry with: - Pathophysiology descriptions with causal chains - Gene/protein annotations (HGNC, GO terms) - Phenotype associations (HP terms) with frequencies - Cell type involvement (CL terms) - Anatomical locations (UBERON terms) - Chemical entities (CHEBI terms) - Treatment annotations (MAXO terms) - Evidence items with PMIDs and exact abstract quotes - Epidemiology, prognosis, diagnostic, and prevention information - Animal model descriptions with phenotype recapitulation details

Vogt–Koyanagi–Harada (VKH) Disease — Comprehensive Disease Characteristics Report (Autoimmune)

Target disease

  • Disease name: Vogt–Koyanagi–Harada disease (VKH)
  • Category: Autoimmune, multisystem inflammatory disease
  • MONDO ID: MONDO_0018092 (OpenTargets disease record) (OpenTargets Search: Vogt-Koyanagi-Harada disease)

Executive summary

VKH is a T cell–driven autoimmune disorder targeting melanocyte-associated antigens in melanin-containing tissues (uvea/choroid, meninges, inner ear, skin/hair), presenting most prominently as bilateral granulomatous uveitis with characteristic choroidal inflammation and exudative retinal detachment, and variably neurologic/auditory and integumentary manifestations. Contemporary management emphasizes early, aggressive systemic corticosteroids combined with early immunomodulatory therapy to prevent progression to chronically evolving disease with “sunset glow fundus” and recurrent inflammation; biologics (e.g., anti‑TNF) and newer small molecules (e.g., JAK inhibitors) are increasingly used for refractory disease. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, rahman2023immunosuppressivetherapyfor pages 1-2, vitale2024efficacyandsafety pages 1-2)

1. Disease information

1.1 Definition and current understanding

A recent 2024 editorial defines VKH as “a multisystemic autoimmune disorder that affects the eyes, central nervous system, the auditory system, and the integumentary system,” driven by an autoimmune reaction against melanocyte-associated antigens across these tissues. (Published online 2024‑04‑24; URL: https://doi.org/10.1080/09273948.2024.2331401) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)

A 2023 COVID-era review similarly describes VKH as “a rare multisystem inflammatory autoimmune disease affecting eyes, ears, brain, skin and hair,” and frames the core immunopathology as T‑cell mediated autoimmunity directed against choroidal melanocytes and melanocyte antigens (e.g., tyrosinase, TRP1/2, MART‑1, gp100). (Published 2023‑09; URL: https://doi.org/10.3390/jcm12196242) (manni2023vogtkoyanagiharadadiseaseand pages 1-2)

1.2 Key identifiers

  • MONDO: MONDO_0018092 (OpenTargets Search: Vogt-Koyanagi-Harada disease)
  • MeSH / ICD‑10 / ICD‑11 / Orphanet: Not extractable from the retrieved full texts in this run; VKH is present as a MeSH term in ClinicalTrials.gov metadata for VKH-related studies (NCT05590416 chunk 2).

1.3 Synonyms / alternative names

  • Vogt–Koyanagi–Harada disease
  • Vogt–Koyanagi–Harada syndrome
  • Harada disease (often used in ophthalmology contexts) (Use of “syndrome” is explicit in multiple sources) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, vitale2024efficacyandsafety pages 1-2)

1.4 Evidence source type

This report is derived from aggregated disease-level resources (reviews/editorials, clinical trial registries) and primary human studies (genetic case–control, randomized trial, retrospective cohorts), plus experimental model systems (rodent and avian models). (zhou2023geneticassociationof pages 1-2, zhong2023arandomizednoninferiority pages 3-4, adamus2002experimentalautoimmuneuveitides pages 1-3, sorrick2022immuneactivitiesin pages 1-2)

2. Etiology

2.1 Disease causal factors (mechanistic)

Core concept: VKH is an autoimmune response against melanocyte-associated antigens.

Human immunology evidence supports antigen-specific T cell reactivity: ocular-infiltrating and peripheral CD4+ T cells from VKH patients recognize melanocyte peptides (tyrosinase and gp100) in an HLA-DR4 (including HLA‑DRB1*0405) restricted manner and produce inflammatory mediators (e.g., IFN‑γ, RANTES). (sugita2006ocularinfiltratingcd4+ pages 1-2, sugita2006ocularinfiltratingcd4+ pages 7-8)

2.2 Risk factors

2.2.1 Genetic risk factors

HLA risk and immunogenetic predisposition - In immune checkpoint inhibitor–associated uveitis, VKH-like presentations were strongly associated with HLA‑DRB1*04:05: “Four patients with VKH-like uveitis underwent HLA genotyping and were all positive for HLA‑DRB104:05… Statistical analysis showed that HLA‑DRB104:05 was significantly associated with developing VKH-like ICIU (P = 0.029).” (Published 2023‑08; URL: https://doi.org/10.1038/s41598-023-40565-z) (takeuchi2023hladrb1*0405isinvolved pages 1-2)

Non-HLA susceptibility loci (recent primary human genetics, 2023) - In a large Chinese Han case–control study (912 VKH, 878 controls), PRKCD and CARD9 polymorphisms were associated with VKH susceptibility. The abstract states: “We found that rs74437127 C allele of PRKCD… and C allele of CARD9 were associated with increased susceptibility of VKH… Functional studies… revealed that CC carriers had significantly higher CARD9 mRNA expression and tumour necrosis factor-α production…” (Published 2023‑02; URL: https://doi.org/10.1186/s40246-023-00459-7) (zhou2023geneticassociationof pages 1-2) - PRKCD rs74437127 C allele: Pc=0.020, OR=1.624 (95% CI 1.200–2.199) (zhou2023geneticassociationof pages 1-2) - CARD9 rs3812555 CC genotype: Pc=2.04×10^-5, OR=1.810 (95% CI 1.418–2.311) (zhou2023geneticassociationof pages 1-2) - Functional correlate: rs3812555 CC carriers had higher CARD9 mRNA and higher TNF‑α production (P=1.00×10^-4; P=2.00×10^-3) (zhou2023geneticassociationof pages 1-2)

2.2.2 Environmental / infectious / iatrogenic triggers

  • Viral triggers and molecular mimicry are repeatedly proposed; in VKH, melanocyte antigen–specific T cells can show cross-reactivity with a CMV peptide, supporting a plausible mimicry mechanism. (sugita2006ocularinfiltratingcd4+ pages 7-8)

SARS‑CoV‑2 infection and COVID‑19 vaccination (2023–2024 focus) - A 2023 review summarizes reported VKH onset/relapse after COVID‑19 infection/vaccination and notes VKH is “one of the most frequently reported uveitic entities after COVID‑19 vaccination,” while emphasizing good response to therapy. (Published 2023‑09; URL: https://doi.org/10.3390/jcm12196242) (manni2023vogtkoyanagiharadadiseaseand pages 1-2) - A focused 2023 case-series review of vaccine-associated VKH included 21 patients and reported a mean onset interval of 7.5 days (range 12 h to 4 weeks), frequent bilateral involvement (20/21), meningitis symptoms (16/21), and frequent serous retinal detachment (16/21) and choroidal thickening (14/21). (Published 2023‑06; URL: https://doi.org/10.1080/21645515.2023.2220630) (manni2023vogtkoyanagiharadadiseaseand pages 2-4) - A 2024 Japanese center study found higher clinic-based prevalence among new patients after the COVID-19 state-of-emergency declaration, but similar visual acuity and recurrence outcomes after pulse steroids. (Published 2024‑06; URL: https://doi.org/10.1038/s41598-024-63957-1) (muto2024effectofthe pages 6-7)

Immune checkpoint inhibitors (ICIs) - ICI-associated uveitis occurs in ~0.3–1% of ICI-treated patients, and VKH-like posterior/panuveitis can occur with choroidal thickening and serous subretinal fluid. (takeuchi2023hladrb1*0405isinvolved pages 1-2)

2.3 Protective factors

No specific genetic or environmental protective factors are established as clinical recommendations; however, specific alleles/genotypes in the PRKCD/CARD9 study were statistically associated with reduced susceptibility (e.g., PRKCD rs74437127 T allele OR=0.616; CARD9 rs3812555 T allele OR=0.589), which may be viewed as candidate protective associations rather than proven protective mechanisms. (zhou2023geneticassociationof pages 1-2)

2.4 Gene–environment interaction

The prevailing model is genetic susceptibility (notably HLA-DR4-related) plus an environmental/infectious/iatrogenic trigger (e.g., viral infection, vaccination, ICI-mediated immune disinhibition) leading to loss of tolerance to melanocyte antigens. This interaction is explicitly invoked in COVID-era synthesis and in ICI-associated VKH-like uveitis. (manni2023vogtkoyanagiharadadiseaseand pages 1-2, takeuchi2023hladrb1*0405isinvolved pages 1-2)

3. Phenotypes (clinical spectrum)

3.1 Clinical stages / temporal development

Four phases are classically described: 1) Prodromal (neurologic/auditory symptoms; CSF pleocytosis), 2) Acute uveitic (diffuse choroiditis → papilledema/exudative detachments; may evolve to panuveitis), 3) Convalescent (depigmentation; vitiligo/poliosis/alopecia), 4) Chronic recurrent (chronic recurrent granulomatous anterior uveitis; less commonly recurrent exudative detachments). (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)

A COVID-era review notes the prodromal phase is often ~3–5 days and the acute uveitic stage lasts weeks with bilateral posterior uveitis and choroidal thickening/serous detachments. (manni2023vogtkoyanagiharadadiseaseand pages 2-4)

3.2 Phenotype frequencies from a large cohort (recent primary data)

In the Chinese Han VKH cohort (n=912), reported frequencies included: uveitis 100%, sunset glow fundus 48.7%, headache 49.2%, tinnitus 45.0%, vitiligo 11.4%, alopecia 31.4%. (zhou2023geneticassociationof pages 1-2)

3.3 Key phenotype types (examples) and suggested ontology terms

Below are representative phenotype mappings (not exhaustive).

Ocular - Bilateral granulomatous panuveitis / diffuse choroiditis (HPO: Uveitis; Choroiditis; Panuveitis). (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, manni2023vogtkoyanagiharadadiseaseand pages 2-4) - Exudative/serous retinal detachment (HPO: Retinal detachment; often “serous retinal detachment”) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, manni2023vogtkoyanagiharadadiseaseand pages 2-4) - Choroidal thickening (HPO candidate: Abnormal choroid morphology / Choroidal thickening). (manni2023vogtkoyanagiharadadiseaseand pages 16-19, tugaltutkun2024vogtkoyanagiharadadisease pages 1-3) - “Sunset glow fundus” (clinical sign; HPO candidate: Abnormal fundus pigmentation). (feng2024predictivefactorsand pages 1-2, zhou2023geneticassociationof pages 1-2)

Neurologic - Headache; aseptic meningitis / meningeal symptoms (HPO: Headache; Meningitis; Cerebrospinal fluid pleocytosis) (manni2023vogtkoyanagiharadadiseaseand pages 2-4, zhou2023geneticassociationof pages 1-2)

Auditory - Tinnitus (HPO: Tinnitus) (zhou2023geneticassociationof pages 1-2)

Integumentary - Vitiligo, poliosis, alopecia (HPO: Vitiligo; Poliosis; Alopecia) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, zhou2023geneticassociationof pages 1-2)

3.4 Anatomical structures affected (UBERON suggestions)

  • Eye uvea/choroid (UBERON: uvea, choroid) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)
  • Meninges / CNS coverings (UBERON: meninges) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)
  • Inner ear / auditory system (UBERON: inner ear) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)
  • Skin and hair follicles (UBERON: skin, hair follicle) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)

3.5 Cell types involved (CL suggestions)

  • CD4+ T cell (CL: T-helper cell) (sugita2006ocularinfiltratingcd4+ pages 1-2)
  • Th1‑polarized effector/memory CD4+ T cells (CL: effector memory T cell; functional subtype) (sugita2006ocularinfiltratingcd4+ pages 7-8)
  • Macrophage (CL: macrophage) (supported by animal-model infiltrates) (sorrick2022immuneactivitiesin pages 1-2)
  • B cell (CL: B cell) (supported by animal-model infiltrates and B-cell involvement in chronic disease) (sorrick2022immuneactivitiesin pages 1-2, elasrar2021newperspectiveson pages 1-2)

4. Genetic / molecular information

4.1 “Causal” genes

VKH is not a Mendelian disorder with a single causal gene in the retrieved evidence; it is best characterized as multifactorial/polygenic with strong HLA associations and multiple immune pathway loci.

4.2 Susceptibility variants (recent primary evidence)

  • PRKCD rs74437127: C allele risk (OR 1.624) and T allele protective (OR 0.616) (zhou2023geneticassociationof pages 1-2)
  • CARD9 rs3812555: CC genotype risk (OR 1.810), C allele risk (OR 1.698), with functional association to higher CARD9 mRNA and TNF‑α. (zhou2023geneticassociationof pages 1-2)

4.3 Molecular pathophysiology (causal chain)

1) Predisposition (e.g., HLA‑DRB104:05 and other genetic factors) → 2) Trigger (viral infection/vaccination or iatrogenic immune disinhibition with ICIs) → 3) Antigen presentation and loss of tolerance to melanocyte antigens (e.g., tyrosinase/gp100) → 4) Effector inflammation in choroid/uvea with cytokine production (TNF‑α, IFN‑γ; Th1/Th17 signatures) → 5) Tissue damage and melanocyte loss* leading to ocular exudation/detachments acutely and depigmentation (sunset glow fundus; vitiligo/poliosis/alopecia) chronically. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, sugita2006ocularinfiltratingcd4+ pages 1-2, zhou2023geneticassociationof pages 1-2, manni2023vogtkoyanagiharadadiseaseand pages 1-2)

4.4 GO biological process suggestions

  • T cell activation; antigen processing and presentation (MHC class II); cytokine-mediated signaling; inflammatory response; leukocyte migration/chemotaxis (supported by RANTES and IFN‑γ responses) (sugita2006ocularinfiltratingcd4+ pages 7-8, sugita2006ocularinfiltratingcd4+ pages 1-2)

5. Environmental information

Key non-genetic contributors described in the retrieved evidence include viral infections and vaccinations as plausible triggers, particularly highlighted in COVID-era literature, and iatrogenic triggers such as immune checkpoint inhibitors. (manni2023vogtkoyanagiharadadiseaseand pages 1-2, takeuchi2023hladrb1*0405isinvolved pages 1-2)

6. Mechanism / pathophysiology (expanded)

6.1 Immune system involvement

  • VKH is characterized by melanocyte antigen–directed CD4+ T cell responses (Th1-like functional profile) and inflammatory mediator production (e.g., IFN‑γ; RANTES). (sugita2006ocularinfiltratingcd4+ pages 1-2, sugita2006ocularinfiltratingcd4+ pages 7-8)
  • CARD9 risk genotype correlates with increased TNF‑α production, linking innate/adaptive signaling nodes to inflammatory cytokine output in VKH susceptibility. (zhou2023geneticassociationof pages 1-2)

6.2 Expert synthesis (therapeutic window concept)

A 2021 mechanistic treatment review argues that early high-dose corticosteroids alone may be insufficient to prevent chronic evolution and emphasizes early combination therapy (e.g., corticosteroids + mycophenolate mofetil) as a “window of opportunity” to prevent chronically evolving disease; it also highlights B cell involvement in chronic disease, supported by responsiveness to rituximab in refractory cases. (URL: https://doi.org/10.3389/fmed.2021.705796; publication 2021‑11) (elasrar2021newperspectiveson pages 1-2)

7. Anatomical structures affected

Primary involved structures include the uveal tract (notably choroid), with systemic involvement in meninges, inner ear, and integumentary tissues (skin/hair) as described in modern reviews and classification discussions. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, manni2023vogtkoyanagiharadadiseaseand pages 1-2)

8. Temporal development

  • Typical onset: Most commonly in adults (2nd–5th decades), with prodromal systemic symptoms possible before ocular disease. (manni2023vogtkoyanagiharadadiseaseand pages 1-2, manni2023vogtkoyanagiharadadiseaseand pages 2-4)
  • Course: May resolve with early therapy or evolve into chronically recurrent disease with anterior granulomatous uveitis and depigmentation. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)

9. Inheritance and population

9.1 Inheritance

Best classified as multifactorial/polygenic, with strong HLA class II associations and additional immune gene contributions (PRKCD/CARD9). (zhou2023geneticassociationof pages 1-2, takeuchi2023hladrb1*0405isinvolved pages 1-2)

9.2 Population demographics / geographic distribution

  • VKH is more frequent in pigmented populations (e.g., Asians, Middle Easterners, Hispanics, Native Americans) and relatively rare in Europe; women often predominate and onset is typically in the 2nd–5th decades. (manni2023vogtkoyanagiharadadiseaseand pages 1-2, li2023bibliometricanalysisof pages 1-2)

9.3 Recent epidemiology statistics available in retrieved literature

  • Puerto Rico cohort (n=24) suggested seasonality: 50% onset in fall vs 12.5% in spring (P=0.043). (Published 2023‑02; URL: https://doi.org/10.1080/09273948.2022.2029499) (amaral2023; retrieved but not fully evidence-scanned in this run)
  • True population incidence/prevalence per 100,000 for VKH specifically was not obtained from the retrieved full texts; available numbers in retrieved COVID-era review include vaccine-induced uveitis incidence (8–13/100,000/year) but this is not VKH-specific. (manni2023vogtkoyanagiharadadiseaseand pages 2-4)

10. Diagnostics

10.1 Clinical diagnostic criteria and recent comparisons

Diagnosis is clinical with multimodal imaging support; multiple criteria sets are discussed. In a Chinese case–control comparison summarized in 2024, sensitivities differed substantially: CDCV 92.2%, RDC 66.7%, SUN-C 54.3%, with high specificity across sets. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, tugaltutkun2024vogtkoyanagiharadadisease media 234f6a18, tugaltutkun2024vogtkoyanagiharadadisease media dc9f8533)

10.2 Imaging and laboratory findings (real-world implementation)

Imaging hallmarks in acute disease include choroidal thickening and subretinal fluid on OCT/EDI‑OCT, pinpoint hyperfluorescence/leakage patterns on fluorescein angiography, and hypocfluorescent dots/patches on ICGA. (manni2023vogtkoyanagiharadadiseaseand pages 16-19)

OCTA monitoring (recent development) - In an acute VKH series summarized in 2024, 93.8% of eyes had “dark foci”/flow voids in choriocapillaris and Sattler’s layer at presentation, decreasing with therapy and reduced choroidal thickness, supporting OCTA as a noninvasive monitoring tool. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3, tugaltutkun2024vogtkoyanagiharadadisease media 234f6a18, tugaltutkun2024vogtkoyanagiharadadisease media dc9f8533)

CSF pleocytosis may be present in prodromal/neurologic phase and was reported in vaccine-associated cases (CSF pleocytosis in 7/21 in one review). (manni2023vogtkoyanagiharadadiseaseand pages 2-4)

10.3 Differential diagnosis

Not systematically extracted in the retrieved texts; the 2024 editorial notes OCTA patterns may help differentiate atypical VKH from entities such as APMPPE in some contexts. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)

11. Outcome / prognosis

11.1 Prognostic factors (2024 cohort data)

A 2024 retrospective cohort (Beijing Tongren Hospital; 62 patients, 2020–2023) identified factors associated with chronic recurrence: - Chronic-recurrent group had worse initial BCVA (1.38±0.54 vs 0.64±0.29 logMAR; P=0.002) and higher sunset glow fundus prevalence (64.3% vs 23.5%; P=0.001). (Published 2024‑06; URL: https://doi.org/10.1186/s12886-024-03511-9) (feng2024predictivefactorsand pages 1-2) - Logistic regression predictors included older age at onset (P=0.042) and sunset glow fundus (P=0.037). (feng2024predictivefactorsand pages 1-2)

11.2 COVID/vaccine-associated prognosis

A 2023 synthesis reports favorable short-term outcomes in reported COVID infection/vaccination-associated cases, with high corticosteroid responsiveness and mean visual acuity ~20/32 in short-term follow-up. (manni2023vogtkoyanagiharadadiseaseand pages 20-22)

12. Treatment (current practice, evidence, and trials)

12.1 Standard pharmacotherapy and strategy

A 2023 retrospective series abstract states: “Treatment is usually initiated with corticosteroids followed by an early introduction of immunosuppressive treatment (IMT).” (URL: https://doi.org/10.1186/s12348-023-00333-6; publication 2023‑05) (rahman2023immunosuppressivetherapyfor pages 1-2)

Evidence for early combination IMT - In 26 patients (20-year retrospective), 81% (21/26) treated with combined IMT/low-dose steroids achieved disease stability with improved median VA from 0.3 logMAR to 0.0 logMAR at 24 months (p=0.0001). MMF was commonly used but 50% of MMF-treated patients did not achieve disease control. (rahman2023immunosuppressivetherapyfor pages 1-2)

12.2 Biologics and comparative effectiveness (high-impact 2023 randomized trial)

A 26-week randomized non-inferiority trial (ChiCTR2100043061; 110 randomized) compared a cyclosporine-based immunosuppressant strategy vs an adalimumab-based biologic strategy (both with corticosteroids). The abstract states: “we assigned 110 patients… to cyclosporine-based immunosuppressant strategy… or adalimumab-based biologic strategy… The primary outcome is change from baseline in best-corrected visual acuity at week 26… P < 0.001 for non-inferiority.” (Published 2023‑06; URL: https://doi.org/10.1038/s41467-023-39483-5) (zhong2023arandomizednoninferiority pages 1-2) Key quantitative outcomes: - BCVA improvement: 11.2 letters (95% CI 7.5–14.9) vs 6.3 letters (3.1–9.6); difference 4.9 (0.2–9.5) with one-sided P<0.001 for non-inferiority. (zhong2023arandomizednoninferiority pages 1-2) - Serious adverse events: 0.70 vs 1.21 events per patient-year (lower in cyclosporine strategy). (zhong2023arandomizednoninferiority pages 1-2)

12.3 Adalimumab in chronic-recurrent VKH (2024 observational evidence)

Adalimumab significantly reduced anterior chamber and vitreous inflammatory cells and reduced recurrence rate (P=0.009) in a 2024 retrospective cohort of chronic-recurrent VKH. (feng2024predictivefactorsand pages 1-2)

12.4 JAK inhibitors (recent real-world prospective registry cohort, 2024)

A prospective AIDA network cohort (n=12; included 1 VKH case) found complete ocular control in 12/12 after starting JAK inhibitors and a marked reduction in flare incidence from 125 to 28.6 episodes per 1,000 person-months (incidence rate ratio 4.37; 95% CI 1.3–14.7; p=0.02). (Published 2024‑08‑23; URL: https://doi.org/10.3389/fmed.2024.1439338) (vitale2024efficacyandsafety pages 1-2)

12.5 Clinical trials (real-world implementations and ongoing studies)

  • NCT03399175 (University of São Paulo; recruiting): early high-dose corticosteroid + immunosuppressive therapy with multimodal imaging and functional testing; primary outcome includes scotopic ERG variation (6–12 months). (NCT03399175 chunk 1)
  • URL: https://clinicaltrials.gov/study/NCT03399175 (NCT03399175 chunk 1)
  • NCT05590416 (Tianjin Medical University; recruiting): prospective cohort comparing adalimumab + glucocorticoids vs traditional therapy in acute VKH (onset <1 month); primary outcomes include logMAR BCVA change and recurrence rate at 24 weeks. (NCT05590416 chunk 1)
  • URL: https://clinicaltrials.gov/study/NCT05590416 (NCT05590416 chunk 1)

12.6 MAXO (Medical Action Ontology) term suggestions (representative)

  • Systemic glucocorticoid therapy
  • Immunosuppressive therapy / immunomodulatory therapy (e.g., mycophenolate mofetil, azathioprine, cyclosporine)
  • Tumor necrosis factor inhibitor therapy (adalimumab)
  • Janus kinase inhibitor therapy (baricitinib, tofacitinib, upadacitinib)
  • Multimodal ocular imaging (OCT, ICGA, FA, OCTA) for monitoring (these actions are supported as real-world implementations in the cited studies) (rahman2023immunosuppressivetherapyfor pages 1-2, vitale2024efficacyandsafety pages 1-2, tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)

13. Prevention

13.1 Primary prevention

No established primary prevention exists for VKH in the retrieved evidence.

13.2 Secondary/tertiary prevention (preventing chronic evolution and complications)

The actionable prevention strategy is early recognition and prompt systemic therapy to prevent chronic recurrence and sunset glow fundus; multiple sources emphasize early treatment improves outcomes. (rahman2023immunosuppressivetherapyfor pages 1-2, manni2023vogtkoyanagiharadadiseaseand pages 8-10)

13.3 Vaccination considerations (COVID era)

Despite case reports of VKH onset/relapse after vaccination, expert synthesis emphasizes that good therapeutic response and benefit–risk considerations should not discourage vaccination, while recommending vigilance and early treatment in predisposed subjects. (manni2023vogtkoyanagiharadadiseaseand pages 1-2, manni2023vogtkoyanagiharadadiseaseand pages 20-22)

14. Other species / natural disease

A spontaneous autoimmune pigmentation disorder model (Smyth line chicken) shows systemic melanocyte autoimmunity with ocular involvement resembling VKH and sympathetic ophthalmia; infiltrating leukocytes include CD4+, CD8+ T cells, B cells, and macrophages, and cytokine profiles suggest Th1 polarization. (Published 2022‑04; URL: https://doi.org/10.3389/fmed.2022.846100) (sorrick2022immuneactivitiesin pages 1-2)

15. Model organisms

15.1 Induced experimental VKH-like uveitis models

  • Experimental melanin-protein induced uveitis (EMIU) and related models show that immunization with tyrosinase-related proteins 1 and 2 in rats can produce VKH-like inflammatory changes, supporting melanocyte antigens as mechanistic drivers and providing a platform for antigen-specific therapy concepts. (adamus2002experimentalautoimmuneuveitides pages 1-3)

15.2 Spontaneous model relevant to VKH mechanisms

  • Smyth line chicken autoimmune vitiligo with uveitis/vision impairment offers a spontaneous model to interrogate systemic anti-melanocyte immunity and timing of immune infiltration preceding overt disease. (sorrick2022immuneactivitiesin pages 1-2)

Recent developments and latest research highlights (2023–2024 prioritized)

1) Diagnostic criteria modernization and imaging-based definition of choroidal involvement: CDCV criteria sensitivity 92.2% vs RDC 66.7% vs SUN-C 54.3% in a Chinese case-control comparison summarized in 2024, with explicit incorporation of EDI-OCT/ICGA in some modern criteria. (2024‑04‑24; https://doi.org/10.1080/09273948.2024.2331401) (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3) 2) COVID-19 era trigger literature: systematic compilation of post-infection and post-vaccine VKH, with rapid onset intervals and high corticosteroid responsiveness, supporting continued surveillance rather than a change in standard management. (2023‑09; https://doi.org/10.3390/jcm12196242) (manni2023vogtkoyanagiharadadiseaseand pages 1-2) 3) Head-to-head strategy trial evidence: cyclosporine-based strategy non-inferior to adalimumab-based strategy for 26-week visual improvement, informing real-world decision-making when selecting conventional immunosuppression vs biologics. (2023‑06; https://doi.org/10.1038/s41467-023-39483-5) (zhong2023arandomizednoninferiority pages 1-2) 4) Newer therapeutics: prospective registry evidence supporting JAK inhibitors for refractory non-infectious ocular inflammation including VKH. (2024‑08‑23; https://doi.org/10.3389/fmed.2024.1439338) (vitale2024efficacyandsafety pages 1-2)

Data table of high-yield facts

Domain Specific finding (with numbers where available) Source (first author year journal) Publication date URL Evidence context ID
Identifiers/Definition VKH is a multisystem autoimmune disorder affecting the eyes, central nervous system, auditory system, and integumentary system; disease course includes prodromal, acute uveitic, convalescent, and chronic recurrent phases. Tugal-Tutkun 2024 Ocular Immunology and Inflammation 2024-04-24 https://doi.org/10.1080/09273948.2024.2331401 (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)
Genetics In 912 VKH cases vs 878 controls, PRKCD rs74437127 C allele increased susceptibility (Pc=0.020, OR=1.624, 95% CI 1.200–2.199), while T allele was protective (Pc=0.020, OR=0.616, 95% CI 0.455–0.833). Zhou 2023 Human Genomics 2023-02 https://doi.org/10.1186/s40246-023-00459-7 (zhou2023geneticassociationof pages 1-2)
Genetics CARD9 rs3812555 CC genotype and C allele increased VKH susceptibility (Pc=2.04×10^-5, OR=1.810, 95% CI 1.418–2.311; Pc=2.76×10^-5, OR=1.698, 95% CI 1.362–2.118), whereas TC genotype and T allele were protective (Pc=7.85×10^-5, OR=0.559; Pc=2.76×10^-5, OR=0.589). CC carriers had higher CARD9 mRNA and TNF-α production (P=1.00×10^-4; P=2.00×10^-3). Zhou 2023 Human Genomics 2023-02 https://doi.org/10.1186/s40246-023-00459-7 (zhou2023geneticassociationof pages 1-2)
Phenotypes & staging In the 912-patient VKH cohort, phenotype frequencies were: uveitis 100%, sunset glow fundus 48.7%, headache 49.2%, tinnitus 45.0%, vitiligo 11.4%, alopecia 31.4%. Zhou 2023 Human Genomics 2023-02 https://doi.org/10.1186/s40246-023-00459-7 (zhou2023geneticassociationof pages 1-2)
Genetics / Triggers In immune checkpoint inhibitor-associated uveitis, 5/9 cases were VKH-like and 4/9 non-VKH-like; among genotyped patients, 4/4 VKH-like were HLA-DRB1*04:05 positive vs 0/3 non-VKH-like, with significant association (P=0.029). Uveitis incidence in ICI-treated patients is reported as ~0.3–1%. Takeuchi 2023 Scientific Reports 2023-08 https://doi.org/10.1038/s41598-023-40565-z (takeuchi2023hladrb1*0405isinvolved pages 1-2)
Triggers Proposed triggers include viral infection and vaccination; review identified 4 young post-COVID infection VKH cases (all female; mean age 30 ± 5.43 years; mean onset ~19.8 days after infection), and summarized 33 new-onset post-vaccine cases. Manni 2023 Journal of Clinical Medicine 2023-09 https://doi.org/10.3390/jcm12196242 (manni2023vogtkoyanagiharadadiseaseand pages 8-10, manni2023vogtkoyanagiharadadiseaseand pages 16-19)
Triggers For COVID-19 vaccine-associated VKH review, 21 patients were identified (9 male, 12 female; median age 45 years, range 19–78); 14/21 after first dose, 8/21 after second; mean interval to symptoms 7.5 days; 20/21 bilateral; 16 had meningitis symptoms; 16 serous retinal detachment; 14 choroidal thickening; all received corticosteroids; mean recovery time 2 months. Xu 2023 Human Vaccines & Immunotherapeutics 2023-06 https://doi.org/10.1080/21645515.2023.2220630 (manni2023vogtkoyanagiharadadiseaseand pages 2-4)
Phenotypes & staging Typical acute features include bilateral choroiditis/panuveitis, exudative retinal detachment, meningismus/CSF pleocytosis, auditory and integumentary signs. Prodromal phase lasts ~3–5 days before acute uveitic stage developing over weeks. Manni 2023 Journal of Clinical Medicine 2023-09 https://doi.org/10.3390/jcm12196242 (manni2023vogtkoyanagiharadadiseaseand pages 2-4)
Diagnostics/Imaging Comparison of criteria in Chinese case-control study: Chinese Diagnostic Criteria for VKH (CDCV) sensitivity 92.2%, vs Revised Diagnostic Criteria (RDC) 66.7% and SUN classification criteria 54.3%; all three had high specificity without significant differences. Tugal-Tutkun 2024 Ocular Immunology and Inflammation 2024-04-24 https://doi.org/10.1080/09273948.2024.2331401 (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)
Diagnostics/Imaging For acute VKH monitoring with OCTA, 93.8% of eyes had scattered dark foci in choriocapillaris and Sattler’s layer at presentation; follow-up in 30 eyes showed reduction in dark foci with decreasing choroidal thickness after treatment. Tugal-Tutkun 2024 Ocular Immunology and Inflammation 2024-04-24 https://doi.org/10.1080/09273948.2024.2331401 (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3)
Treatment In a 26-patient retrospective VKH series, treatment shifted from steroid monotherapy to combined immunosuppressive therapy (IMT)/low-dose steroid. 81% (21/26) treated with combined IMT/steroid achieved disease stability with improved median VA from 0.3 logMAR to 0.0 logMAR at 24 months (p=0.0001). Rahman 2023 Journal of Ophthalmic Inflammation and Infection 2023-05 https://doi.org/10.1186/s12348-023-00333-6 (rahman2023immunosuppressivetherapyfor pages 1-2)
Treatment In the same series, MMF monotherapy was most common (13/19 IMT-treated; 68%) and was well tolerated, but 50% of MMF-treated patients did not achieve disease control. Average time from diagnosis to IMT initiation was 2.1 months; average time to steroid-sparing effect was 5 months. Rahman 2023 Journal of Ophthalmic Inflammation and Infection 2023-05 https://doi.org/10.1186/s12348-023-00333-6 (rahman2023immunosuppressivetherapyfor pages 1-2)
Prognosis / Prognostic factors In 62 VKH patients (34 acute-resolved, 28 chronic-recurrent), chronic-recurrent patients were older (49.00 ± 16.43 vs 38.29 ± 15.46 years) and had worse initial BCVA (1.38 ± 0.54 vs 0.64 ± 0.29 logMAR; P=0.002). Complications occurred in 41.7% vs 29.4% (P=0.006), and sunset glow fundus in 64.3% vs 23.5% (P=0.001). Feng 2024 BMC Ophthalmology 2024-06 https://doi.org/10.1186/s12886-024-03511-9 (feng2024predictivefactorsand pages 1-2)
Prognosis / Prognostic factors Predictors of progression to chronic-recurrent VKH included poor initial BCVA (P=0.046) and sunset glow fundus (P=0.040); logistic regression identified older age at onset (P=0.042) and sunset glow fundus (P=0.037) as significant predictors. Feng 2024 BMC Ophthalmology 2024-06 https://doi.org/10.1186/s12886-024-03511-9 (feng2024predictivefactorsand pages 1-2)
Treatment In chronic-recurrent VKH, adalimumab significantly reduced anterior chamber inflammatory cells (P=0.000), vitreous inflammatory cells (P=0.001), and recurrence rate (P=0.009). Feng 2024 BMC Ophthalmology 2024-06 https://doi.org/10.1186/s12886-024-03511-9 (feng2024predictivefactorsand pages 1-2)
Treatment In a prospective AIDA network cohort of 12 adults with non-infectious ocular inflammatory disease (including 1 VKH case), 4 received baricitinib, 1 tofacitinib, and 7 upadacitinib; mean treatment duration was 8.6 ± 5.5 months. Vitale 2024 Frontiers in Medicine 2024-08-23 https://doi.org/10.3389/fmed.2024.1439338 (vitale2024efficacyandsafety pages 1-2)
Treatment With JAK inhibitors, ocular disease control was complete in 12/12 patients; flare incidence fell from 125 to 28.6 episodes per 1,000 person-months, incidence rate ratio 4.37 (95% CI 1.3–14.7, p=0.02) for pre- vs post-JAK periods. Vitale 2024 Frontiers in Medicine 2024-08-23 https://doi.org/10.3389/fmed.2024.1439338 (vitale2024efficacyandsafety pages 1-2)
Prognosis / Prognostic factors Early corticosteroid use is associated with better outcomes; rapid early visual acuity improvement predicts better final VA, while greater relapse number is associated with more complications and worse visual prognosis. Manni 2023 Journal of Clinical Medicine 2023-09 https://doi.org/10.3390/jcm12196242 (manni2023vogtkoyanagiharadadiseaseand pages 8-10)
Clinical trials NCT03399175: single-group interventional prospective study, University of São Paulo; 40 participants; started 2015-03-23; recruiting. Tests early high-dose systemic corticosteroid + immunosuppressive therapy in acute VKH with ≥12-month follow-up using FA, ICGA, EDI-OCT, ERG, autofluorescence, perimetry, contrast sensitivity, and QoL. Primary outcome: scotopic ERG variation between 6 and 12 months. Yamamoto 2015 ClinicalTrials.gov 2015-03-23 start; recruiting https://clinicaltrials.gov/study/NCT03399175 (NCT03399175 chunk 1)
Clinical trials NCT05590416: single-center prospective observational cohort, Tianjin Medical University; 15 participants; recruiting; compares adalimumab (80 mg loading, then 40 mg every 2 weeks) + glucocorticoids vs traditional therapy in acute VKH onset <1 month. Primary outcomes: change in logMAR BCVA and recurrence rate at 24 weeks; secondary outcomes include SUN anterior chamber cell grade and prednisone exposure. Zhang 2021 ClinicalTrials.gov 2021-06-01 start; recruiting https://clinicaltrials.gov/study/NCT05590416 (NCT05590416 chunk 1)

Table: This table compiles high-yield, citable findings on Vogt-Koyanagi-Harada disease across genetics, triggers, phenotypes, diagnostics, treatment, prognosis, and ongoing trials. It is designed to support rapid evidence-backed drafting of a comprehensive disease report.

Limitations of this report (evidence gaps from this run)

  • ICD‑10/ICD‑11 and MeSH descriptor IDs were not retrieved from dedicated ontology sources (e.g., ICD/MeSH browsers) within the accessible full texts; the report therefore provides MONDO and trial-level MeSH categorization only. (OpenTargets Search: Vogt-Koyanagi-Harada disease, NCT05590416 chunk 2)
  • True population-based VKH incidence/prevalence (per 100,000) was not captured in the retrieved full texts; additional targeted epidemiology sources would be required.
  • Quality-of-life instrument outcomes (e.g., NEI-VFQ, SF-36) were not available in retrieved papers; only trial registry mentions QoL questionnaires. (NCT03399175 chunk 1)

References

  1. (OpenTargets Search: Vogt-Koyanagi-Harada disease): Open Targets Query (Vogt-Koyanagi-Harada disease, 0 results). Buniello, A. et al. (2025). Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery. Nucleic Acids Research.

  2. (tugaltutkun2024vogtkoyanagiharadadisease pages 1-3): Ilknur Tugal-Tutkun, Derrick P. Smit, Ahmed M. Abu El-Asrar, Carl P. Herbort, and Jennifer E. Thorne. Vogt-koyanagi-harada disease. Ocular Immunology and Inflammation, 32:363-366, Apr 2024. URL: https://doi.org/10.1080/09273948.2024.2331401, doi:10.1080/09273948.2024.2331401. This article has 12 citations and is from a peer-reviewed journal.

  3. (rahman2023immunosuppressivetherapyfor pages 1-2): Najiha Rahman, Jose Carlo M Artiaga, Konstantinos Bouras, Joshua Luis, Angela Rees, and Mark Westcott. Immunosuppressive therapy for vogt-koyanagi-harada disease: a retrospective study and review of literature. Journal of Ophthalmic Inflammation and Infection, May 2023. URL: https://doi.org/10.1186/s12348-023-00333-6, doi:10.1186/s12348-023-00333-6. This article has 24 citations and is from a peer-reviewed journal.

  4. (vitale2024efficacyandsafety pages 1-2): Antonio Vitale, Judith Palacios-Olid, Valeria Caggiano, Gaafar Ragab, José Hernández-Rodríguez, Laura Pelegrín, Germán Mejía-Salgado, Laura Zarate-Pinzón, Stefano Gentileschi, Jurgen Sota, Alex Fonollosa, Ester Carreño, Carla Gaggiano, Rana Hussein Amin, Alberto Balistreri, Javier Narváez, Gian Marco Tosi, Bruno Frediani, Luca Cantarini, Alejandra de-la-Torre, and Claudia Fabiani. Efficacy and safety of janus kinase inhibitors in non-infectious inflammatory ocular diseases: a prospective cohort study from the international aida network registries. Frontiers in Medicine, Aug 2024. URL: https://doi.org/10.3389/fmed.2024.1439338, doi:10.3389/fmed.2024.1439338. This article has 29 citations.

  5. (manni2023vogtkoyanagiharadadiseaseand pages 1-2): Priscilla Manni, Maria Carmela Saturno, and Massimo Accorinti. Vogt-koyanagi-harada disease and covid. Journal of Clinical Medicine, 12:6242, Sep 2023. URL: https://doi.org/10.3390/jcm12196242, doi:10.3390/jcm12196242. This article has 8 citations.

  6. (NCT05590416 chunk 2): Xiaomin Zhang. A Study of Adalimumab in Acute Vogt-Koyanagi-Harada Disease. Tianjin Medical University. 2021. ClinicalTrials.gov Identifier: NCT05590416

  7. (zhou2023geneticassociationof pages 1-2): Chunya Zhou, Shiya Cai, Yuhong Xie, Zhen Zeng, Jun Zhang, Guannan Su, Qiuying Wu, Xingsheng Ye, Qingfeng Cao, Peizeng Yang, and Jianmin Hu. Genetic association of prkcd and card9 polymorphisms with vogt–koyanagi–harada disease in the chinese han population. Human Genomics, Feb 2023. URL: https://doi.org/10.1186/s40246-023-00459-7, doi:10.1186/s40246-023-00459-7. This article has 3 citations and is from a peer-reviewed journal.

  8. (zhong2023arandomizednoninferiority pages 3-4): Zhenyu Zhong, Lingyu Dai, Qiuying Wu, Yu Gao, Yanlin Pu, Guannan Su, Xiaorong Lu, Fuxiang Zhang, Chong Tang, Yao Wang, Chunjiang Zhou, and Peizeng Yang. A randomized non-inferiority trial of therapeutic strategy with immunosuppressants versus biologics for vogt-koyanagi-harada disease. Nature Communications, Jun 2023. URL: https://doi.org/10.1038/s41467-023-39483-5, doi:10.1038/s41467-023-39483-5. This article has 16 citations and is from a highest quality peer-reviewed journal.

  9. (adamus2002experimentalautoimmuneuveitides pages 1-3): GRAZYNA ADAMUS and CHI-CHAO CHAN. Experimental autoimmune uveitides: multiple antigens, diverse diseases. International Reviews of Immunology, 21:209-229, Jan 2002. URL: https://doi.org/10.1080/08830180212068, doi:10.1080/08830180212068. This article has 85 citations and is from a peer-reviewed journal.

  10. (sorrick2022immuneactivitiesin pages 1-2): Jordan Sorrick, Wilson Huett, Kristen A. Byrne, and Gisela F. Erf. Immune activities in choroids of visually impaired smyth chickens with autoimmune vitiligo. Frontiers in Medicine, Apr 2022. URL: https://doi.org/10.3389/fmed.2022.846100, doi:10.3389/fmed.2022.846100. This article has 4 citations.

  11. (sugita2006ocularinfiltratingcd4+ pages 1-2): Sunao Sugita, Hiroshi Takase, Chikako Taguchi, Yasuhisa Imai, Koju Kamoi, Tatsushi Kawaguchi, Yoshiharu Sugamoto, Yuri Futagami, Kyogo Itoh, and Manabu Mochizuki. Ocular infiltrating cd4+ t cells from patients with vogt-koyanagi-harada disease recognize human melanocyte antigens. Investigative ophthalmology & visual science, 47 6:2547-54, Jun 2006. URL: https://doi.org/10.1167/iovs.05-1547, doi:10.1167/iovs.05-1547. This article has 202 citations and is from a domain leading peer-reviewed journal.

  12. (sugita2006ocularinfiltratingcd4+ pages 7-8): Sunao Sugita, Hiroshi Takase, Chikako Taguchi, Yasuhisa Imai, Koju Kamoi, Tatsushi Kawaguchi, Yoshiharu Sugamoto, Yuri Futagami, Kyogo Itoh, and Manabu Mochizuki. Ocular infiltrating cd4+ t cells from patients with vogt-koyanagi-harada disease recognize human melanocyte antigens. Investigative ophthalmology & visual science, 47 6:2547-54, Jun 2006. URL: https://doi.org/10.1167/iovs.05-1547, doi:10.1167/iovs.05-1547. This article has 202 citations and is from a domain leading peer-reviewed journal.

  13. (takeuchi2023hladrb10405isinvolved pages 1-2): Masaki Takeuchi, Akira Meguro, Jutaro Nakamura, Rei Chikagawa, Raiga Osada, Etsuko Shibuya, Yukiko Hasumi, Norihiro Yamada, Mami Ishihara, and Nobuhisa Mizuki. Hla-drb104:05 is involved in the development of vogt–koyanagi–harada disease-like immune-related adverse events in patients receiving immune checkpoint inhibitors. Scientific Reports, Aug 2023. URL: https://doi.org/10.1038/s41598-023-40565-z, doi:10.1038/s41598-023-40565-z. This article has 17 citations and is from a peer-reviewed journal.

  14. (manni2023vogtkoyanagiharadadiseaseand pages 2-4): Priscilla Manni, Maria Carmela Saturno, and Massimo Accorinti. Vogt-koyanagi-harada disease and covid. Journal of Clinical Medicine, 12:6242, Sep 2023. URL: https://doi.org/10.3390/jcm12196242, doi:10.3390/jcm12196242. This article has 8 citations.

  15. (muto2024effectofthe pages 6-7): Tetsuya Muto, Masaaki Sakamoto, Shoichiro Kusuda, Yasuo Haruyama, Shigeki Machida, Shinichiro Imaizumi, and Tetsuju Sekiryu. Effect of the covid-19 pandemic on vogt–koyanagi–harada disease. Scientific Reports, Jun 2024. URL: https://doi.org/10.1038/s41598-024-63957-1, doi:10.1038/s41598-024-63957-1. This article has 5 citations and is from a peer-reviewed journal.

  16. (manni2023vogtkoyanagiharadadiseaseand pages 16-19): Priscilla Manni, Maria Carmela Saturno, and Massimo Accorinti. Vogt-koyanagi-harada disease and covid. Journal of Clinical Medicine, 12:6242, Sep 2023. URL: https://doi.org/10.3390/jcm12196242, doi:10.3390/jcm12196242. This article has 8 citations.

  17. (feng2024predictivefactorsand pages 1-2): Hui Feng, Weixin Chen, Jianzhu Yang, Haorong Kong, Hongyu Li, Yuan He, and Hong Wang. Predictive factors and adalimumab efficacy in managing chronic recurrence vogt-koyanagi-harada disease. BMC Ophthalmology, Jun 2024. URL: https://doi.org/10.1186/s12886-024-03511-9, doi:10.1186/s12886-024-03511-9. This article has 9 citations and is from a peer-reviewed journal.

  18. (elasrar2021newperspectiveson pages 1-2): Ahmed M. Abu El-Asrar, Jo Van Damme, Sofie Struyf, and Ghislain Opdenakker. New perspectives on the immunopathogenesis and treatment of uveitis associated with vogt-koyanagi-harada disease. Frontiers in Medicine, Nov 2021. URL: https://doi.org/10.3389/fmed.2021.705796, doi:10.3389/fmed.2021.705796. This article has 34 citations.

  19. (li2023bibliometricanalysisof pages 1-2): Liangpin Li, Liyun Yuan, Xueyan Zhou, Xia Hua, and Xiaoyong Yuan. Bibliometric analysis of the vogt‒koyanagi‒harada disease literature. International Ophthalmology, 43:4137-4150, Aug 2023. URL: https://doi.org/10.1007/s10792-023-02815-x, doi:10.1007/s10792-023-02815-x. This article has 2 citations and is from a peer-reviewed journal.

  20. (tugaltutkun2024vogtkoyanagiharadadisease media 234f6a18): Ilknur Tugal-Tutkun, Derrick P. Smit, Ahmed M. Abu El-Asrar, Carl P. Herbort, and Jennifer E. Thorne. Vogt-koyanagi-harada disease. Ocular Immunology and Inflammation, 32:363-366, Apr 2024. URL: https://doi.org/10.1080/09273948.2024.2331401, doi:10.1080/09273948.2024.2331401. This article has 12 citations and is from a peer-reviewed journal.

  21. (tugaltutkun2024vogtkoyanagiharadadisease media dc9f8533): Ilknur Tugal-Tutkun, Derrick P. Smit, Ahmed M. Abu El-Asrar, Carl P. Herbort, and Jennifer E. Thorne. Vogt-koyanagi-harada disease. Ocular Immunology and Inflammation, 32:363-366, Apr 2024. URL: https://doi.org/10.1080/09273948.2024.2331401, doi:10.1080/09273948.2024.2331401. This article has 12 citations and is from a peer-reviewed journal.

  22. (manni2023vogtkoyanagiharadadiseaseand pages 20-22): Priscilla Manni, Maria Carmela Saturno, and Massimo Accorinti. Vogt-koyanagi-harada disease and covid. Journal of Clinical Medicine, 12:6242, Sep 2023. URL: https://doi.org/10.3390/jcm12196242, doi:10.3390/jcm12196242. This article has 8 citations.

  23. (zhong2023arandomizednoninferiority pages 1-2): Zhenyu Zhong, Lingyu Dai, Qiuying Wu, Yu Gao, Yanlin Pu, Guannan Su, Xiaorong Lu, Fuxiang Zhang, Chong Tang, Yao Wang, Chunjiang Zhou, and Peizeng Yang. A randomized non-inferiority trial of therapeutic strategy with immunosuppressants versus biologics for vogt-koyanagi-harada disease. Nature Communications, Jun 2023. URL: https://doi.org/10.1038/s41467-023-39483-5, doi:10.1038/s41467-023-39483-5. This article has 16 citations and is from a highest quality peer-reviewed journal.

  24. (NCT03399175 chunk 1): Joyce Hisae Yamamoto. Early Immunosuppressive Therapy on the Course of Vogt-Koyanagi-Harada Disease. University of Sao Paulo. 2015. ClinicalTrials.gov Identifier: NCT03399175

  25. (NCT05590416 chunk 1): Xiaomin Zhang. A Study of Adalimumab in Acute Vogt-Koyanagi-Harada Disease. Tianjin Medical University. 2021. ClinicalTrials.gov Identifier: NCT05590416

  26. (manni2023vogtkoyanagiharadadiseaseand pages 8-10): Priscilla Manni, Maria Carmela Saturno, and Massimo Accorinti. Vogt-koyanagi-harada disease and covid. Journal of Clinical Medicine, 12:6242, Sep 2023. URL: https://doi.org/10.3390/jcm12196242, doi:10.3390/jcm12196242. This article has 8 citations.