Home / Deep Research / IgG4-Related Disease

IgG4-Related Disease

1. Disease Information

2026-05-09
Falcon MONDO:0017287 Model: Edison Scientific Literature 34 citations

1. Disease Information

1.1 Concise overview (current understanding)

IgG4-related disease is a systemic, immune-mediated fibroinflammatory disorder that can produce tumefactive (mass-like) lesions in one or multiple organs and is unified by characteristic histopathology and clinicopathologic correlation (wallace2024currentandfuture pages 2-4, chen2024igg4relateddiseasefor pages 1-2). A core reason IgG4-RD is diagnostically challenging is that no single serologic test is both sensitive and specific, so biopsy and integrative assessment are central (wallace2024currentandfuture pages 2-4, bartoszek2024igg4relateddiseasecomprehensive pages 4-7).

Key diagnostic concept: tissue lesions typically show (i) dense lymphoplasmacytic infiltrate enriched for IgG4+ plasma cells, (ii) storiform fibrosis, and (iii) obliterative phlebitis, often with eosinophilia (chen2024igg4relateddiseasefor pages 1-2, wallace2024currentandfuture pages 2-4).

Direct abstract quote (identifier resource): Pieringer et al. state: “Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder (ORPHA284264).” (pieringer2014igg4relateddisease pages 1-2)

1.2 Synonyms / alternative names

Commonly used alternative phrasings include “IgG4-related disease,” “IgG4-RD,” and organ-specific manifestations (e.g., type 1 autoimmune pancreatitis; IgG4-related sclerosing cholangitis; retroperitoneal fibrosis as a phenotype) (chen2024igg4relateddiseasefor pages 1-2, pieringer2014igg4relateddisease pages 2-4, wallace2024currentandfuture pages 2-4).

1.3 Evidence source type

The information below is derived primarily from aggregated disease-level resources (peer-reviewed reviews and guidelines) and from human cohort studies/registries and clinical trial registry records (wallace2024currentandfuture pages 2-4, wallace2020the2019american pages 1-1, wallace2024currentandfuture pages 1-2, NCT07148791 chunk 2).

2. Etiology

2.1 Disease causal factors (mechanistic)

The aetiology remains incompletely resolved, but convergent evidence indicates a B cell–T cell–driven immune disorder with downstream fibroinflammation (wallace2024currentandfuture pages 2-4, hao2023thespectrumof pages 2-4).

2.2 Risk factors

Genetic risk factors (susceptibility)

  • HLA associations have been reported in IgG4-RD and related phenotypes. Examples in the retrieved corpus include:
  • HLA-DRB1*03 association reported for retroperitoneal fibrosis (RPF) subtype (bartoszek2024igg4relateddiseasecomprehensive pages 4-7).
  • HLA-DR4 proposed susceptibility in autoimmune pancreatitis (AIP; an IgG4-RD manifestation) (wallace2019immunoglobuling4relateddisease. pages 1-2).
  • HLA-DRB1*16 and HLA-DQB1*05 described as “strongly associated” with autoimmune pancreatitis in one cohort (motta2024igg4autoantibodiesand pages 15-15).

Environmental / occupational risk factors

Infectious triggers

No unifying infectious trigger is established in the retrieved evidence; reviews emphasize the absence of a consistent infectious cause (bartoszek2024igg4relateddiseasecomprehensive pages 4-7, wallace2019immunoglobuling4relateddisease. pages 1-2).

2.3 Protective factors

No specific protective genetic or environmental factors were identified in the retrieved corpus.

2.4 Gene–environment interactions

Not clearly established in the retrieved corpus; current data support multifactorial susceptibility with organ-specific exposures and HLA background (bartoszek2024igg4relateddiseasecomprehensive pages 4-7, wallace2019immunoglobuling4relateddisease. pages 1-2).

3. Phenotypes (clinical manifestations)

3.1 Common organ involvement and clinical phenotypes

Contemporary clinical practice recognizes reproducible organ-pattern phenotypes, including: - Pancreato-hepatobiliary (type 1 autoimmune pancreatitis; sclerosing cholangitis) (wallace2024currentandfuture pages 2-4, chen2024igg4relateddiseasefor pages 1-2) - Retroperitoneum/aorta (retroperitoneal fibrosis; aortitis/large-vessel disease) (wallace2024currentandfuture pages 2-4, chen2024igg4relateddiseasefor pages 1-2) - Head-and-neck limited (salivary/lacrimal gland enlargement; orbital adnexal disease) (wallace2024currentandfuture pages 2-4) - Mikulicz/systemic (symmetric lacrimal/salivary enlargement plus systemic involvement; typically very high serum IgG4) (wallace2024currentandfuture pages 2-4)

Multi-organ involvement is common (reported as 60–90% in one review) (bartoszek2024igg4relateddiseasecomprehensive pages 4-7).

3.2 Frequencies and statistics from recent cohorts/surveys

  • In a large international cohort analysis, mean organ involvement was 2.9 organs (SD 1.8), serum IgG4 was elevated in 78.7%, and classic storiform fibrosis was reported in 39.6% (illustrating that classic histology is not always captured in routine reporting) (wallace2019clinicalphenotypesof pages 2-3).
  • In a Japanese nationwide survey focused on IgG4-RD with malignancy, organ frequencies included: autoimmune pancreatitis 44.7%, sialadenitis 20.8%, eye disease 14.0%, kidney disease 5.16%, retroperitoneal fibrosis 5.12% (sumimoto2022nationwideepidemiologicalsurvey pages 1-1).

3.3 Thoracic (lung/mediastinal) phenotype

Thoracic disease is clinically important and often incidental: - Up to 30% of systemic IgG4-RD patients may have thoracic involvement; thoracic disease is the sole manifestation in ~10% (muller2023thoracicmanifestationsof pages 1-2). - Reported estimates across cohorts are ~15–35% thoracic involvement (muller2023thoracicmanifestationsof pages 2-3).

3.4 Suggested HPO terms (examples; not exhaustive)

(These are ontology suggestions for knowledge-base encoding; mapping not directly cited in the retrieved literature.) - Tumefactive lesion / mass effect: HP:0002664 (Neoplasm) (used as proxy for mass-like lesion), HP:0033694 (Organomegaly) depending on organ. - Salivary/lacrimal enlargement: HP:0000217 (Xerostomia), HP:0001097 (Dry eyes), HP:0000175 (Parotid enlargement). - Obstructive jaundice (pancreatobiliary): HP:0000952 (Jaundice). - Retroperitoneal fibrosis-related obstruction: HP:0000795 (Ureteral obstruction). - Eosinophilia: HP:0001880 (Eosinophilia). - Hypergammaglobulinemia: HP:0012204 (Hypergammaglobulinemia).

4. Genetic / Molecular Information

4.1 Causal genes

IgG4-RD is not established as a monogenic disorder in the retrieved evidence. Genetic findings are primarily susceptibility associations (HLA and selected non-HLA loci) rather than causal variants (bartoszek2024igg4relateddiseasecomprehensive pages 4-7, motta2024igg4autoantibodiesand pages 15-15).

4.2 Pathogenic variants

No recurrent pathogenic germline variants defining IgG4-RD were identified in the retrieved corpus.

4.3 Autoantigens / autoantibodies (molecular targets)

Multiple candidate autoantigens have been reported in IgG4-RD. Examples include galectin-3 (reported in 28% of a cohort) and laminin-511 (detected in 50% of subjects with IgG4-related pancreatitis), supporting antigen-driven immunity in at least some patients (wallace2019immunoglobuling4relateddisease. pages 1-2).

5. Environmental Information

Environmental associations appear phenotype-specific: - Occupational exposure to solvents/oils/industrial & metal dusts has been associated with IgG4-related cholangitis/pancreatitis (bartoszek2024igg4relateddiseasecomprehensive pages 4-7). - Smoking and asbestos exposure have been linked to retroperitoneal fibrosis risk (bartoszek2024igg4relateddiseasecomprehensive pages 4-7, wallace2019immunoglobuling4relateddisease. pages 1-2).

No consistent infectious agent trigger is supported in the retrieved evidence (bartoszek2024igg4relateddiseasecomprehensive pages 4-7, wallace2019immunoglobuling4relateddisease. pages 1-2).

6. Mechanism / Pathophysiology (current model)

6.1 Core cellular players (with CL term suggestions)

Evidence converges on immune dysregulation involving: - B-lineage cells, especially plasmablasts (suggest CL:0000946 plasma cell; plasmablasts are a transitional phenotype). - T follicular helper (Tfh) cells (suggest CL:0000907 T follicular helper cell). - CD4+ cytotoxic T lymphocytes (CD4+ CTLs) (suggest CL:0000899 CD4-positive, alpha-beta T cell; “cytotoxic CD4” is a functional state).

6.2 B-cell/plasmablast axis and fibrosis linkage

Circulating plasmablasts are an important mechanistic and biomarker node: plasmablast levels correlate with serum IgG4, inflammatory markers, number of involved organs, and disease activity, and they fall with effective therapy and rise with relapse (hao2023thespectrumof pages 2-4, hao2023thespectrumof pages 4-6). A mechanistic link to fibrosis is proposed through plasmablast profibrotic programs (e.g., LOXL2 expression and PDGF-B secretion) (hao2023thespectrumof pages 2-4).

6.3 Tfh-driven class switching and plasmablast generation

Tfh cells provide B-cell help and promote IgG4 class-switching via IL-4/IL-21 and co-stimulatory interactions. Circulating PD-1+ Tfh measures correlate with serum IgG/IgG4 metrics and the number of organs involved (xu2024pathogenicrolesof pages 2-4). A 2024 review explicitly notes that “the expansion of circulating Tfh2 cells and plasmablasts may also serve as novel biomarkers for disease diagnosis and activity monitoring” (xu2024pathogenicrolesof pages 1-2).

6.4 CD4+ CTLs and profibrotic mediators

CD4+ CTLs infiltrate lesions and can produce profibrotic mediators such as TGF-β, providing a plausible bridge between immune activation and storiform fibrosis (hao2023thespectrumof pages 2-4).

6.5 Suggested GO biological process terms (examples)

(These are ontology suggestions; not directly asserted as GO annotations in the retrieved papers.) - GO:0006954 inflammatory response - GO:0042113 B cell activation - GO:0002460 adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains - GO:0001525 angiogenesis / vascular remodeling (for obliterative phlebitis context) - GO:0042060 wound healing and GO:0006959 humoral immune response - GO:0045429 positive regulation of nitric oxide biosynthetic process (macrophage-related; hypothesis-level)

6.6 Suggested causal chain (integrated)

A plausible chain supported by current evidence is: antigen-driven or dysregulated immune activation → expansion of Tfh (especially Tfh2-like) and plasmablasts → IgG4 class switching and oligoclonal plasmablast expansion → recruitment/activation of profibrotic T-cell and myeloid programs (including CD4+ CTLs) → storiform fibrosis and organ dysfunction (hao2023thespectrumof pages 2-4, xu2024pathogenicrolesof pages 2-4, wallace2024currentandfuture pages 2-4).

7. Anatomical Structures Affected

7.1 Organs and systems (examples)

IgG4-RD can affect virtually any organ; commonly involved sites include pancreas and hepatobiliary system, salivary/lacrimal glands and orbit, retroperitoneum/aorta, kidney, lung/mediastinum, and lymph nodes (chen2024igg4relateddiseasefor pages 1-2, pieringer2014igg4relateddisease pages 2-4, wallace2024currentandfuture pages 2-4).

7.2 UBERON suggestions (examples)

8. Temporal Development

8.1 Onset

IgG4-RD typically affects middle-aged to older adults (median around late 50s–60 in major cohorts) (chen2024igg4relateddiseasefor pages 1-2, muller2023thoracicmanifestationsof pages 2-3).

8.2 Course and progression

The disease often follows a relapsing–remitting course; if under-treated, it can cause irreversible fibrotic organ damage (wallace2024currentandfuture pages 2-4, chen2024igg4relateddiseasefor pages 1-2). Proliferative manifestations tend to respond better than chronic fibrotic disease, which may be less reversible (chen2024igg4relateddiseasefor pages 1-2).

9. Inheritance and Population

9.1 Epidemiology (recent estimates prioritized)

A 2024 practice review summarized a US claims-based analysis reporting: - Incidence: 0.78–1.39 per 100,000 person-years (2015–2019) - Point prevalence: 5.3 per 100,000 persons (as of 1 Jan 2019) (wallace2024currentandfuture pages 1-2)

A thoracic-manifestations review summarizes additional estimates, e.g., Japan incidence approximated at ~1 per 100,000 and prevalence ~0.8 per 100,000 (muller2023thoracicmanifestationsof pages 2-3), while also emphasizing heterogeneity.

9.2 Demographics

10. Diagnostics

10.1 Histopathology and core features

Hallmark tissue features include dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis, obliterative phlebitis, and eosinophilia. A common supportive threshold is an IgG4/IgG ratio >40% in tissue (chen2024igg4relateddiseasefor pages 1-2).

10.2 Classification criteria (2019 ACR/EULAR)

The 2019 ACR/EULAR criteria provide a validated, points-based framework requiring: entry organ involvement, exclusion criteria application, and weighted inclusion domains across clinical/serologic/radiologic/pathologic data, with classification at ≥20 points (wallace2020the2019american pages 1-1). Performance: - Derived/validated in 1,879 subjects (1,086 cases; 793 mimickers) (wallace2020the2019american pages 1-1) - Validation cohort 1: specificity 99.2%, sensitivity 85.5% at threshold 20 (wallace2020the2019american pages 1-1) - Validation cohort 2: specificity 97.8%, sensitivity 82.0% at threshold 20 (wallace2020the2019american pages 1-1)

10.3 Biomarkers

10.4 Imaging and diagnostic pitfalls

Thoracic IgG4-RD has heterogeneous CT patterns and can mimic malignancy or vasculitis; cautious diagnosis includes searching for extra-thoracic disease and integrating serum IgG4/plasmablast measures plus pathology (muller2023thoracicmanifestationsof pages 1-2).

11. Outcome / Prognosis

11.1 Organ damage and reversibility

Clinical outcomes vary by phenotype: proliferative manifestations generally respond well to immunosuppression, whereas established fibrotic disease may be less reversible (chen2024igg4relateddiseasefor pages 1-2).

11.2 Malignancy association (statistics)

A Japanese nationwide survey reported an estimated overall prevalence of malignancy among IgG4-RD cases of ~10.9% (10,900 per 100,000 cases), and malignant lymphoma ~2.0% (1,985 per 100,000). IgG4-related kidney disease had the highest associated malignancy frequency (17.1%) (sumimoto2022nationwideepidemiologicalsurvey pages 1-1). These data are context-specific (survey methodology) and should be interpreted accordingly.

12. Treatment

12.1 Standard of care: glucocorticoids (real-world implementation)

European evidence-based guidance for IgG4-related digestive disease recommends: - Induction: oral glucocorticoids 0.6–0.8 mg/kg/day for 1 month (typical 30–40 mg/day prednisone equivalent) (lohr2020europeanguidelineon pages 17-19, lohr2020europeanguidelineon pages 1-2) - Response assessment: at week 2–4 using clinical/biochemical/morphological markers (lohr2020europeanguidelineon pages 17-19, lohr2020europeanguidelineon pages 1-2) - Taper: e.g., 5 mg every 2 weeks, over 3–6 months (lohr2020europeanguidelineon pages 17-19) - Relapse: common; guideline notes relapse rates 26–70% and high re-induction success with glucocorticoids (>95%) (lohr2020europeanguidelineon pages 19-20)

12.2 B-cell depletion and steroid-sparing therapy

12.3 Emerging and investigational immunomodulators (clinical trials)

Note: A placebo-controlled inebilizumab study is referenced in a 2024 table, but the excerpt available here is truncated and does not provide full registry detail (wallace2024currentandfuture pages 7-8).

12.4 MAXO term suggestions (examples)

  • Glucocorticoid therapy: MAXO:0000155 (glucocorticoid therapy)
  • B-cell depletion therapy (rituximab): MAXO:0001181 (B cell depletion therapy) (term may vary)
  • Abatacept therapy: MAXO: immunomodulatory therapy
  • Belimumab therapy: MAXO: monoclonal antibody therapy
  • CAR-T cell therapy: MAXO:0001503 (CAR T cell therapy)

13. Prevention

Primary prevention

No established primary prevention strategies are supported by the retrieved evidence.

Secondary/tertiary prevention (practical)

14. Other Species / Natural Disease

No evidence for naturally occurring IgG4-RD in non-human species was retrieved in this run.

15. Model Organisms

No standardized animal model systems were retrieved in the accessible corpus. A mouse immunization experiment linked to laminin-511 is mentioned as supporting an antigen-driven mechanism but details were not available in the retrieved text segments (wallace2019immunoglobuling4relateddisease. pages 1-2).

Key curated summary table

Table (click to expand)
Category Item Summary Publication URL Citation
Identifier Orphanet ID IgG4-related disease is identified as a rare systemic fibro-inflammatory disorder with Orphanet identifier ORPHA284264. Pieringer et al., 2014 https://doi.org/10.1186/s13023-014-0110-z (pieringer2014igg4relateddisease pages 1-2)
Defining histopathology Lymphoplasmacytic infiltrate rich in IgG4+ plasma cells Hallmark lesion includes a dense lymphoplasmacytic infiltrate enriched in IgG4-positive plasma cells; tissue diagnosis is essential. Chen, 2024 https://doi.org/10.1182/hematology.2024000584 (chen2024igg4relateddiseasefor pages 1-2)
Defining histopathology Storiform fibrosis Storiform fibrosis is a core histopathologic feature and part of the classic triad used in diagnosis/classification. Chen, 2024 https://doi.org/10.1182/hematology.2024000584 (chen2024igg4relateddiseasefor pages 1-2)
Defining histopathology Obliterative phlebitis Obliterative phlebitis is a characteristic histologic feature supporting IgG4-RD diagnosis. Chen, 2024 https://doi.org/10.1182/hematology.2024000584 (chen2024igg4relateddiseasefor pages 1-2)
Supportive finding Tissue eosinophilia Tissue eosinophilia is a common supportive feature accompanying the histopathologic triad. Chen, 2024 https://doi.org/10.1182/hematology.2024000584 (chen2024igg4relateddiseasefor pages 1-2)
Supportive finding Serum IgG4 elevation Many patients have elevated serum IgG4; this correlates with extent of organ involvement and relapse risk, but is not specific enough to diagnose disease alone. Wallace et al., 2024 https://doi.org/10.1093/rap/rkae020 (wallace2024currentandfuture pages 2-4)
Supportive finding Tissue IgG4/IgG ratio A commonly used supportive tissue threshold is an IgG4:IgG plasma-cell ratio >40%. Chen, 2024 https://doi.org/10.1182/hematology.2024000584 (chen2024igg4relateddiseasefor pages 1-2)
Classification criteria 2019 ACR/EULAR entry and threshold Classification uses a 3-step process (typical organ involvement, exclusion criteria, weighted inclusion criteria) with a threshold of ≥20 points. Wallace et al., 2020 https://doi.org/10.1136/annrheumdis-2019-216561 (wallace2020the2019american pages 1-1)
Classification performance Cohort size used in development/validation Criteria were developed/validated using 1,879 subjects total (1,086 cases and 793 mimickers). Wallace et al., 2020 https://doi.org/10.1136/annrheumdis-2019-216561 (wallace2020the2019american pages 1-1)
Classification performance Validation cohort 1 At ≥20 points, specificity was 99.2% (95% CI 97.2–99.8) and sensitivity was 85.5% (95% CI 81.9–88.5). Wallace et al., 2020 https://doi.org/10.1136/annrheumdis-2019-216561 (wallace2020the2019american pages 1-1)
Classification performance Validation cohort 2 At ≥20 points, specificity was 97.8% (95% CI 93.7–99.2) and sensitivity was 82.0% (95% CI 77.0–86.1). Wallace et al., 2020 https://doi.org/10.1136/annrheumdis-2019-216561 (wallace2020the2019american pages 1-1)

Table: This table summarizes the core disease identifier, defining pathology, supportive findings, and validated 2019 ACR/EULAR classification performance for IgG4-related disease. It is useful as a compact reference for knowledge-base curation and diagnostic context.

Notes on evidence gaps and limitations

  1. Ontology identifiers (MONDO, MeSH, ICD) were not retrievable from the current tool-accessible literature corpus; the report therefore does not assert them.
  2. Several important 2023–2025 primary studies (e.g., single-cell transcriptomics; some therapeutics RCT publications) were listed as unobtainable in tool retrieval; mechanistic and treatment sections thus emphasize accessible, high-quality reviews/cohorts and clinical trial registry records.
  3. HPO/GO/UBERON/MAXO terms are provided as suggestions for encoding and were not directly mapped in the cited papers.

References

  1. (pieringer2014igg4relateddisease pages 1-2): Herwig Pieringer, Ilse Parzer, Adelheid Wöhrer, Petra Reis, Bastian Oppl, and Jochen Zwerina. Igg4- related disease: an orphan disease with many faces. Orphanet Journal of Rare Diseases, 9:110-110, Jul 2014. URL: https://doi.org/10.1186/s13023-014-0110-z, doi:10.1186/s13023-014-0110-z. This article has 142 citations and is from a peer-reviewed journal.

  2. (wallace2024currentandfuture pages 2-4): Zachary S Wallace, Guy Katz, Yasmin G Hernandez-Barco, and Matthew C Baker. Current and future advances in practice: igg4-related disease. Rheumatology Advances in Practice, Apr 2024. URL: https://doi.org/10.1093/rap/rkae020, doi:10.1093/rap/rkae020. This article has 71 citations and is from a peer-reviewed journal.

  3. (chen2024igg4relateddiseasefor pages 1-2): Luke Y. C. Chen. Igg4-related disease for the hematologist. Hematology, 2024:594-603, Dec 2024. URL: https://doi.org/10.1182/hematology.2024000584, doi:10.1182/hematology.2024000584. This article has 15 citations and is from a peer-reviewed journal.

  4. (bartoszek2024igg4relateddiseasecomprehensive pages 4-7): Lidia Bartoszek, Dominika Orłowska, Joanna Olszak, Karolina Zalewa, Wojciech Kapłan, Jakub Starownik, and Bartłomiej Gastoł. Igg4-related disease: comprehensive overview of pathogenesis, clinical manifestations, and diagnostic challenges. Quality in Sport, 22:54293, Sep 2024. URL: https://doi.org/10.12775/qs.2024.22.54293, doi:10.12775/qs.2024.22.54293. This article has 0 citations.

  5. (pieringer2014igg4relateddisease pages 2-4): Herwig Pieringer, Ilse Parzer, Adelheid Wöhrer, Petra Reis, Bastian Oppl, and Jochen Zwerina. Igg4- related disease: an orphan disease with many faces. Orphanet Journal of Rare Diseases, 9:110-110, Jul 2014. URL: https://doi.org/10.1186/s13023-014-0110-z, doi:10.1186/s13023-014-0110-z. This article has 142 citations and is from a peer-reviewed journal.

  6. (wallace2020the2019american pages 1-1): Zachary S Wallace, Ray P Naden, Suresh Chari, Hyon K Choi, Emanuel Della-Torre, Jean-Francois Dicaire, Phillip A Hart, Dai Inoue, Mitsuhiro Kawano, Arezou Khosroshahi, Marco Lanzillotta, Kazuichi Okazaki, Cory A Perugino, Amita Sharma, Takako Saeki, Nicolas Schleinitz, Naoki Takahashi, Hisanori Umehara, Yoh Zen, and John H Stone. The 2019 american college of rheumatology/european league against rheumatism classification criteria for igg4-related disease. Annals of the Rheumatic Diseases, 79:77-87, Jan 2020. URL: https://doi.org/10.1136/annrheumdis-2019-216561, doi:10.1136/annrheumdis-2019-216561. This article has 1362 citations and is from a highest quality peer-reviewed journal.

  7. (wallace2024currentandfuture pages 1-2): Zachary S Wallace, Guy Katz, Yasmin G Hernandez-Barco, and Matthew C Baker. Current and future advances in practice: igg4-related disease. Rheumatology Advances in Practice, Apr 2024. URL: https://doi.org/10.1093/rap/rkae020, doi:10.1093/rap/rkae020. This article has 71 citations and is from a peer-reviewed journal.

  8. (NCT07148791 chunk 2): Jian Zhu. Exploratory Study of Anti-BCMA-CD19 CAR-T Cell Therapy in Relapsed or Refractory IgG4-Related Disease. Chinese PLA General Hospital. 2025. ClinicalTrials.gov Identifier: NCT07148791

  9. (hao2023thespectrumof pages 2-4): Qiyuan Hao, Meng Sun, and Yanying Liu. The spectrum of b cells in the pathogenesis, diagnosis and therapeutic applications of immunoglobulin g4‐related disease. Clinical & Translational Immunology, Jan 2023. URL: https://doi.org/10.1002/cti2.1477, doi:10.1002/cti2.1477. This article has 7 citations and is from a peer-reviewed journal.

  10. (wallace2019immunoglobuling4relateddisease. pages 1-2): Zachary S. Wallace, Cory Perugino, Mark Matza, Vikram Deshpande, Amita Sharma, and John H. Stone. Immunoglobulin g4-related disease. Clinics in chest medicine, 40 3:583-597, Sep 2019. URL: https://doi.org/10.1016/j.ccm.2019.05.005, doi:10.1016/j.ccm.2019.05.005. This article has 70 citations and is from a peer-reviewed journal.

  11. (motta2024igg4autoantibodiesand pages 15-15): Rodrigo V. Motta and Emma L. Culver. Igg4 autoantibodies and autoantigens in the context of igg4-autoimmune disease and igg4-related disease. Frontiers in Immunology, Feb 2024. URL: https://doi.org/10.3389/fimmu.2024.1272084, doi:10.3389/fimmu.2024.1272084. This article has 30 citations and is from a peer-reviewed journal.

  12. (wallace2019clinicalphenotypesof pages 2-3): Zachary S Wallace, Yuqing Zhang, Cory A Perugino, Ray Naden, Hyon K Choi, and John H Stone. Clinical phenotypes of igg4-related disease: an analysis of two international cross-sectional cohorts. Annals of the Rheumatic Diseases, 78:406-412, Mar 2019. URL: https://doi.org/10.1136/annrheumdis-2018-214603, doi:10.1136/annrheumdis-2018-214603. This article has 422 citations and is from a highest quality peer-reviewed journal.

  13. (sumimoto2022nationwideepidemiologicalsurvey pages 1-1): Kimi Sumimoto, Kazushige Uchida, Tsukasa Ikeura, Kenji Hirano, Motohisa Yamamoto, Hiroki Takahashi, Takayoshi Nishino, Ichiro Mizushima, Mitsuhiro Kawano, Terumi Kamisawa, Takako Saeki, Hiroyuki Maguchi, Tomoyuki Ushijima, Masahiro Shiokawa, Hiroshi Seno, Hiroshi Goto, Seiji Nakamura, and Kazuichi Okazaki. Nationwide epidemiological survey of immunoglobulin g4‐related disease with malignancy in japan. Journal of Gastroenterology and Hepatology, 37:1022-1033, Mar 2022. URL: https://doi.org/10.1111/jgh.15809, doi:10.1111/jgh.15809. This article has 26 citations and is from a peer-reviewed journal.

  14. (muller2023thoracicmanifestationsof pages 1-2): Romain Muller, Mikael Ebbo, Paul Habert, Laurent Daniel, Antoine Briantais, Pascal Chanez, Jean Yves Gaubert, and Nicolas Schleinitz. Thoracic manifestations of igg4‐related disease. Respirology (Carlton, Vic.), 28:120-131, Nov 2023. URL: https://doi.org/10.1111/resp.14422, doi:10.1111/resp.14422. This article has 58 citations.

  15. (muller2023thoracicmanifestationsof pages 2-3): Romain Muller, Mikael Ebbo, Paul Habert, Laurent Daniel, Antoine Briantais, Pascal Chanez, Jean Yves Gaubert, and Nicolas Schleinitz. Thoracic manifestations of igg4‐related disease. Respirology (Carlton, Vic.), 28:120-131, Nov 2023. URL: https://doi.org/10.1111/resp.14422, doi:10.1111/resp.14422. This article has 58 citations.

  16. (hao2023thespectrumof pages 4-6): Qiyuan Hao, Meng Sun, and Yanying Liu. The spectrum of b cells in the pathogenesis, diagnosis and therapeutic applications of immunoglobulin g4‐related disease. Clinical & Translational Immunology, Jan 2023. URL: https://doi.org/10.1002/cti2.1477, doi:10.1002/cti2.1477. This article has 7 citations and is from a peer-reviewed journal.

  17. (xu2024pathogenicrolesof pages 2-4): Jingyi Xu, Jiayu Zhai, and Jinxia Zhao. Pathogenic roles of follicular helper t cells in igg4-related disease and implications for potential therapy. Frontiers in Immunology, Jun 2024. URL: https://doi.org/10.3389/fimmu.2024.1413860, doi:10.3389/fimmu.2024.1413860. This article has 18 citations and is from a peer-reviewed journal.

  18. (xu2024pathogenicrolesof pages 1-2): Jingyi Xu, Jiayu Zhai, and Jinxia Zhao. Pathogenic roles of follicular helper t cells in igg4-related disease and implications for potential therapy. Frontiers in Immunology, Jun 2024. URL: https://doi.org/10.3389/fimmu.2024.1413860, doi:10.3389/fimmu.2024.1413860. This article has 18 citations and is from a peer-reviewed journal.

  19. (lohr2020europeanguidelineon pages 17-19): J‐Matthias Löhr, Ulrich Beuers, Miroslav Vujasinovic, Domenico Alvaro, Jens Brøndum Frøkjær, Frank Buttgereit, Gabriele Capurso, Emma L Culver, Enrique de‐Madaria, Emanuel Della‐Torre, Sönke Detlefsen, Enrique Dominguez‐Muñoz, Piotr Czubkowski, Nils Ewald, Luca Frulloni, Natalya Gubergrits, Deniz Guney Duman, Thilo Hackert, Julio Iglesias‐Garcia, Nikolaos Kartalis, Andrea Laghi, Frank Lammert, Fredrik Lindgren, Alexey Okhlobystin, Grzegorz Oracz, Andrea Parniczky, Raffaella Maria Pozzi Mucelli, Vinciane Rebours, Jonas Rosendahl, Nicolas Schleinitz, Alexander Schneider, Eric FH van Bommel, Caroline Sophie Verbeke, Marie Pierre Vullierme, and Heiko Witt. European guideline on igg4‐related digestive disease – ueg and sgf evidence‐based recommendations. United European Gastroenterology Journal, 8:637-666, Jul 2020. URL: https://doi.org/10.1177/2050640620934911, doi:10.1177/2050640620934911. This article has 272 citations and is from a peer-reviewed journal.

  20. (lohr2020europeanguidelineon pages 1-2): J‐Matthias Löhr, Ulrich Beuers, Miroslav Vujasinovic, Domenico Alvaro, Jens Brøndum Frøkjær, Frank Buttgereit, Gabriele Capurso, Emma L Culver, Enrique de‐Madaria, Emanuel Della‐Torre, Sönke Detlefsen, Enrique Dominguez‐Muñoz, Piotr Czubkowski, Nils Ewald, Luca Frulloni, Natalya Gubergrits, Deniz Guney Duman, Thilo Hackert, Julio Iglesias‐Garcia, Nikolaos Kartalis, Andrea Laghi, Frank Lammert, Fredrik Lindgren, Alexey Okhlobystin, Grzegorz Oracz, Andrea Parniczky, Raffaella Maria Pozzi Mucelli, Vinciane Rebours, Jonas Rosendahl, Nicolas Schleinitz, Alexander Schneider, Eric FH van Bommel, Caroline Sophie Verbeke, Marie Pierre Vullierme, and Heiko Witt. European guideline on igg4‐related digestive disease – ueg and sgf evidence‐based recommendations. United European Gastroenterology Journal, 8:637-666, Jul 2020. URL: https://doi.org/10.1177/2050640620934911, doi:10.1177/2050640620934911. This article has 272 citations and is from a peer-reviewed journal.

  21. (lohr2020europeanguidelineon pages 19-20): J‐Matthias Löhr, Ulrich Beuers, Miroslav Vujasinovic, Domenico Alvaro, Jens Brøndum Frøkjær, Frank Buttgereit, Gabriele Capurso, Emma L Culver, Enrique de‐Madaria, Emanuel Della‐Torre, Sönke Detlefsen, Enrique Dominguez‐Muñoz, Piotr Czubkowski, Nils Ewald, Luca Frulloni, Natalya Gubergrits, Deniz Guney Duman, Thilo Hackert, Julio Iglesias‐Garcia, Nikolaos Kartalis, Andrea Laghi, Frank Lammert, Fredrik Lindgren, Alexey Okhlobystin, Grzegorz Oracz, Andrea Parniczky, Raffaella Maria Pozzi Mucelli, Vinciane Rebours, Jonas Rosendahl, Nicolas Schleinitz, Alexander Schneider, Eric FH van Bommel, Caroline Sophie Verbeke, Marie Pierre Vullierme, and Heiko Witt. European guideline on igg4‐related digestive disease – ueg and sgf evidence‐based recommendations. United European Gastroenterology Journal, 8:637-666, Jul 2020. URL: https://doi.org/10.1177/2050640620934911, doi:10.1177/2050640620934911. This article has 272 citations and is from a peer-reviewed journal.

  22. (NCT01584388 chunk 2): John H. Stone, MD. Rituximab in IgG4-RD: A Phase 1-2 Trial. Massachusetts General Hospital. 2012. ClinicalTrials.gov Identifier: NCT01584388

  23. (wallace2024currentandfuture pages 7-8): Zachary S Wallace, Guy Katz, Yasmin G Hernandez-Barco, and Matthew C Baker. Current and future advances in practice: igg4-related disease. Rheumatology Advances in Practice, Apr 2024. URL: https://doi.org/10.1093/rap/rkae020, doi:10.1093/rap/rkae020. This article has 71 citations and is from a peer-reviewed journal.

  24. (NCT03669861 chunk 2): Stone, John H, M.D., M.P.H. Safety and Efficacy of Abatacept in IgG4-Related Disease. Massachusetts General Hospital. 2018. ClinicalTrials.gov Identifier: NCT03669861