A chronic autoimmune connective tissue disease characterized by vasculopathy, immune dysregulation, and progressive fibrosis of the skin and internal organs. Classified into limited cutaneous and diffuse cutaneous subtypes based on extent of skin involvement.
Systemic sclerosis is a multisystem autoimmune disease defined by a triad of early microvasculopathy, immune dysregulation with disease-specific autoantibodies and type I interferon (IFN-I) activation, and progressive fibroblast activation culminating in tissue fibrosis. Recent work confirms that defective angiogenesis and endothelial injury are early, often pre-fibrotic features; “defective angiogenesis … represents a hallmark of early-stage disease, usually preceding the onset of tissue fibrosis” and involves endothelial apoptosis, senescence, and endothelial-to-mesenchymal transition (EndoMT) with impaired pro-angiogenic signaling (e.g., dysregulated VEGF/Angiopoietin axes) (Published Jun 2024; https://doi.org/10.3390/biomedicines12061331) (romano2024recentinsightsinto pages 17-18). Contemporary reviews capturing 2023 advances reiterate the canonical sequence—microvascular damage, immune activation/autoantibodies, then fibrosis—while adding single-cell and genetic resolution of pathway heterogeneity across autoantibody subsets (Published Apr 2024; https://doi.org/10.55563/clinexprheumatol/is29he) (lepri2024systemicsclerosisone pages 1-2).
At the immune level, IFN-I pathway activation (pDC/monocyte signatures, SIGLEC1 upregulation) and B-cell abnormalities are prominent; B-cell/autoantibody phenotypes correlate with organ involvement, including active, immunoglobulin-secreting anti–topoisomerase I responses that associate with interstitial lung disease (ILD) severity (Published Jul 2023; https://doi.org/10.1136/rmdopen-2023-003148) (lepri2024systemicsclerosisone pages 1-2). Vascular and immune perturbations converge on fibroblasts via profibrotic signaling nodes—TGF-β/SMAD, PDGF/CTGF, Wnt, and Hippo (YAP/TAZ)—with recent single-cell analyses in 2024 linking Hippo/YAP–TAZ activity to myofibroblast and EndoMT trajectories in SSc skin (Published Jan 2024; https://doi.org/10.1038/s41467-023-44645-6) (lepri2024systemicsclerosisone pages 1-2). Metabolic reprogramming in stromal and immune compartments (glycolysis/lactate, TCA/OXPHOS shifts) is increasingly implicated, aligning with metabolomic fingerprints (amino acid, acylcarnitine, and TCA intermediates) that distinguish subtypes and pulmonary complications (Published Aug 2023; https://doi.org/10.3389/fmolb.2023.1215039) (maggio2023biomarkersinsystemic pages 6-8).
Genetically, large-scale GWAS (2024) identified six novel SSc susceptibility loci in a Japanese cohort (1,428 cases; 112,599 controls), including a lead signal in the FCGR/FCRL region and context-specific interaction with IRF8-related variants; cross-ancestry meta-analysis added 30 loci, emphasizing roles for B-cell biology and IRF8 in susceptibility (Published Jan 2024; https://doi.org/10.1038/s41467-023-44541-z) (lepri2024systemicsclerosisone pages 1-2). Clinically, these mechanisms underlie organ complications: ILD and pulmonary arterial hypertension (PAH) remain leading drivers of morbidity and mortality, while cardiac involvement (arrhythmia, myocardial fibrosis) and scleroderma renal crisis reflect microvascular injury, immune activation, and fibrosis interplay (Published Aug 2024; https://doi.org/10.1007/s00296-024-05699-x; Published Apr 2024; https://doi.org/10.3390/ijms25094728) (lepri2024systemicsclerosisone pages 1-2).
| HGNC symbol | Name | Role in SSc (summary) | Key pathway (GO term suggestion) | Cellular component (GO-CC) | Cell types (CL terms) | Anatomical sites (UBERON) | Notable molecules/chemicals (CHEBI) | Recent sources (URL, year) |
|---|---|---|---|---|---|---|---|---|
| TGFB1 | Transforming growth factor beta 1 | Central profibrotic cytokine driving fibroblast-to-myofibroblast transition and ECM deposition in skin and lung fibrosis | TGF-beta receptor signaling pathway (GO:0007179) | Extracellular region / secreted (GO:0005576) | Fibroblast (CL:0000064), endothelial cell (CL:0000115) | Skin (UBERON:0002097), Lung (UBERON:0002048) | TGF-β1 (CHEBI:TGF-β1) | https://doi.org/10.1007/s10238-022-00841-0 (2023) (jimenez2025areviewof pages 1-2, maggio2023biomarkersinsystemic pages 6-8) |
| TGFBR1 / TGFBR2 | TGF-β receptors type I & II | Receptors mediating SMAD phosphorylation and downstream profibrotic transcriptional program in SSc fibroblasts | TGF-beta receptor signaling pathway (GO:0007179) | Plasma membrane (GO:0005886) | Fibroblast (CL:0000064), Endothelial cell (CL:0000115) | Skin, Lung | — (receptor complex) | https://doi.org/10.1007/s10238-022-00841-0 (2023) (jimenez2025areviewof pages 1-2) |
| SMAD2 / SMAD3 | SMAD family members 2 & 3 | Intracellular mediators of canonical TGF-β signaling promoting collagen gene expression in SSc fibroblasts | Positive regulation of transcription by SMAD (GO:0060395) | Nucleus / cytoplasm (GO:0005634 / GO:0005737) | Fibroblast (CL:0000064) | Skin, Lung | p-SMAD2/3 (CHEBI:phosphoprotein) | https://doi.org/10.3389/fimmu.2025.1551911 (2025) (jimenez2025areviewof pages 1-2, jimenez2025areviewof pages 9-10) |
| CCN2 (CTGF) | Connective tissue growth factor | Matricellular effector induced by TGF-β that amplifies fibroblast activation and matrix deposition | Regulation of cell proliferation and ECM organization (GO:0030198) | Extracellular matrix (GO:0031012) | Fibroblast (CL:0000064) | Skin, Heart, Lung | CTGF/CCN2 (CHEBI:CTGF) | https://doi.org/10.3389/fimmu.2025.1551911 (2025) (jimenez2025areviewof pages 1-2) |
| PDGFA / PDGFRB | Platelet-derived growth factor A / receptor β | Stimulates fibroblast proliferation and perivascular smooth muscle activation contributing to vascular remodeling and fibrosis | PDGF receptor signaling pathway (GO:0048008) | Plasma membrane / extracellular (GO:0005886 / GO:0005576) | Fibroblast, Pericyte, Vascular smooth muscle cell | Skin, Lung, Vasculature | PDGF-BB (CHEBI:PDGF) | https://doi.org/10.3389/fimmu.2025.1551911 (2025) (jimenez2025areviewof pages 1-2) |
| EDN1 | Endothelin 1 (endothelin-1) | Vasoconstrictor elevated in SSc vasculopathy; implicated in vessel tone dysregulation and may link to fibrosis | Endothelin signaling (GO:0007186) | Secreted peptide (GO:0005576) | Endothelial cell (CL:0000115), VSMC | Peripheral vasculature, Lung | Endothelin-1 (CHEBI:ET-1) | https://doi.org/10.3390/cimb45100490 (2023) (maggio2023biomarkersinsystemic pages 2-4) |
| VEGFA | Vascular endothelial growth factor A | Dysregulated/ectopic VEGF expression: early elevated VEGF but defective angiogenesis and capillary loss in SSc | VEGF receptor signaling pathway (GO:0048010) | Secreted / extracellular (GO:0005576) | Endothelial cell (CL:0000115), Endothelial progenitor cell | Skin microvasculature, Lung | VEGF-A (CHEBI:VEGF) | https://doi.org/10.3390/cimb45100490 (2023), https://doi.org/10.3390/biomedicines12061331 (2024) (maggio2023biomarkersinsystemic pages 6-8, romano2024recentinsightsinto pages 17-18) |
| ANGPT1 / ANGPT2 | Angiopoietin-1 / -2 | Imbalanced Ang1 (decreased) / Ang2 (increased) axis contributes to aberrant angiogenesis and vessel instability in SSc | Angiopoietin-Tie signaling (GO:0043066) | Extracellular region / secreted (GO:0005576) | Endothelial cell (CL:0000115), Pericyte | Microvasculature (skin) | Angiopoietin-2 (CHEBI:ANGPT2) | https://doi.org/10.3390/cimb45100490 (2023) (maggio2023biomarkersinsystemic pages 6-8) |
| CXCL4 | C-X-C motif chemokine ligand 4 (platelet factor 4) | Proposed DAMP / biomarker; promotes IFN-I and inflammatory signaling and associates with lung fibrosis and worse skin scores | Chemokine-mediated signaling pathway (GO:0070098) | Extracellular region / platelet granule (GO:0005576) | Platelet, Plasmacytoid dendritic cell, Monocyte | Skin, Lung | CXCL4 (CHEBI:CXCL4) | https://doi.org/10.3390/cimb45100490 (2023), https://doi.org/10.3390/ijms26062421 (2025) (maggio2023biomarkersinsystemic pages 6-8, bazso2025theroleof pages 1-2) |
| IL6 | Interleukin-6 | Pro-inflammatory/profibrotic cytokine; correlates with mRSS and progressive skin disease and ILD risk | JAK-STAT signaling pathway (GO:0043401) | Secreted cytokine (GO:0005576) | Macrophage, T cell, Fibroblast | Skin, Lung | IL-6 (CHEBI:IL6) | https://doi.org/10.3390/cimb45100490 (2023) (maggio2023biomarkersinsystemic pages 6-8) |
| CCL2 | C-C motif chemokine ligand 2 (MCP-1) | Monocyte chemoattractant linked to macrophage recruitment and ILD / skin severity in SSc | Monocyte chemotaxis (GO:0002548) | Extracellular region (GO:0005576) | Monocyte, Macrophage | Lung, Skin | CCL2 (CHEBI:CCL2) | https://doi.org/10.3390/cimb45100490 (2023) (maggio2023biomarkersinsystemic pages 6-8) |
| IRF8 | Interferon regulatory factor 8 | Genetic susceptibility locus; regulator of IFN-I responses and B cell / myeloid programs implicated in SSc risk and B cell biology | Regulation of type I interferon production (GO:0032479) | Nucleus (GO:0005634) | B cell (CL:0000236), Dendritic cell (CL:0000451), Monocyte | Blood / Immune system | IRF8 (CHEBI:IRF8) | https://doi.org/10.1038/s41467-023-44541-z (2024) (lepri2024systemicsclerosisone pages 1-2) |
| FCGR cluster (e.g., FCGR2A/FCGR3A) | Fc gamma receptors IIa / IIIa region | GWAS locus (FCGR/FCRL region) with strong association in Asian cohort; implicates B cell/FC receptor–mediated immunity in SSc susceptibility | Fc-gamma receptor signaling (GO:0038094) | Plasma membrane (GO:0005886) | B cell, NK cell, Macrophage | Immune system compartments | IgG / immune complexes (CHEBI:IgG) | https://doi.org/10.1038/s41467-023-44541-z (2024) (lepri2024systemicsclerosisone pages 1-2) |
| YAP1 / WWTR1 (TAZ) | YAP1 and WWTR1 (TAZ) Hippo pathway effectors | Hippo/YAP-TAZ signaling implicated in mesenchymal differentiation and myofibroblast/EndoMT fibrotic programs in SSc skin | Hippo signaling (GO:0035329) | Nucleus / cytoplasm (GO:0005634 / GO:0005737) | Fibroblast (CL:0000064), Endothelial-to-mesenchymal cells | Skin | YAP/TAZ transcriptional coactivators (CHEBI:YAP) | https://doi.org/10.1038/s41467-023-44645-6 (2024) (lepri2024systemicsclerosisone pages 1-2) |
| WNT5A | Wnt family member 5A | Non-canonical Wnt implicated in fibroblast activation, crosstalk with Hippo and TGF-β pathways | Wnt signaling pathway (GO:0016055) | Secreted signaling molecule (GO:0005576) | Fibroblast, Endothelial cell | Skin, Lung | Wnt5a (CHEBI:WNT5A) | https://doi.org/10.55563/clinexprheumatol/is29he (2024) (lepri2024systemicsclerosisone pages 1-2) |
| SIGLEC1 | Sialic acid binding Ig-like lectin 1 (CD169) | IFN-I–inducible marker (monocyte/macrophage) used as surrogate of IFN activation in SSc; links IFN axis to immune activation | Type I interferon signaling pathway (GO:0060337) | Plasma membrane (GO:0005886) | Monocyte / Macrophage (CL:0000235) | Blood, Skin | SIGLEC1 (CHEBI:SIGLEC1) | https://doi.org/10.3390/cimb45100490 (2023) (maggio2023biomarkersinsystemic pages 6-8) |
Table: Concise table mapping principal genes/proteins implicated in systemic sclerosis to roles, suggested GO pathways, cellular components, cell types, anatomical sites, notable chemicals, and recent sources (2023–2025) for rapid reference.
References
(romano2024recentinsightsinto pages 17-18): Eloisa Romano, Irene Rosa, Bianca Saveria Fioretto, and Mirko Manetti. Recent insights into cellular and molecular mechanisms of defective angiogenesis in systemic sclerosis. Biomedicines, 12:1331, Jun 2024. URL: https://doi.org/10.3390/biomedicines12061331, doi:10.3390/biomedicines12061331. This article has 13 citations and is from a poor quality or predatory journal.
(lepri2024systemicsclerosisone pages 1-2): Gemma Lepri, Marco Di Battista, Veronica Codullo, Francesco Bonomi, Antonello Sulis, Serena Guiducci, and Alessandra Della Rossa. Systemic sclerosis: one year in review 2024. Clinical and experimental rheumatology, Apr 2024. URL: https://doi.org/10.55563/clinexprheumatol/is29he, doi:10.55563/clinexprheumatol/is29he. This article has 25 citations and is from a peer-reviewed journal.
(maggio2023biomarkersinsystemic pages 6-8): Giuseppe Di Maggio, Paola Confalonieri, Francesco Salton, Liliana Trotta, Luca Ruggero, Metka Kodric, Pietro Geri, Michael Hughes, Mattia Bellan, Michele Gilio, Selene Lerda, Elisa Baratella, Marco Confalonieri, Lucrezia Mondini, and Barbara Ruaro. Biomarkers in systemic sclerosis: an overview. Current Issues in Molecular Biology, 45:7775-7802, Sep 2023. URL: https://doi.org/10.3390/cimb45100490, doi:10.3390/cimb45100490. This article has 25 citations and is from a poor quality or predatory journal.
(maggio2023biomarkersinsystemic pages 2-4): Giuseppe Di Maggio, Paola Confalonieri, Francesco Salton, Liliana Trotta, Luca Ruggero, Metka Kodric, Pietro Geri, Michael Hughes, Mattia Bellan, Michele Gilio, Selene Lerda, Elisa Baratella, Marco Confalonieri, Lucrezia Mondini, and Barbara Ruaro. Biomarkers in systemic sclerosis: an overview. Current Issues in Molecular Biology, 45:7775-7802, Sep 2023. URL: https://doi.org/10.3390/cimb45100490, doi:10.3390/cimb45100490. This article has 25 citations and is from a poor quality or predatory journal.
(jimenez2025areviewof pages 1-2): Sergio A. Jimenez, Fabian A. Mendoza, and Sonsoles Piera-Velazquez. A review of recent studies on the pathogenesis of systemic sclerosis: focus on fibrosis pathways. Frontiers in Immunology, Apr 2025. URL: https://doi.org/10.3389/fimmu.2025.1551911, doi:10.3389/fimmu.2025.1551911. This article has 17 citations and is from a peer-reviewed journal.
(jimenez2025areviewof pages 9-10): Sergio A. Jimenez, Fabian A. Mendoza, and Sonsoles Piera-Velazquez. A review of recent studies on the pathogenesis of systemic sclerosis: focus on fibrosis pathways. Frontiers in Immunology, Apr 2025. URL: https://doi.org/10.3389/fimmu.2025.1551911, doi:10.3389/fimmu.2025.1551911. This article has 17 citations and is from a peer-reviewed journal.
(bazso2025theroleof pages 1-2): Anna Bazsó, Péter Szodoray, Yehuda Shoenfeld, and Emese Virág Kiss Med Dsci. The role of biomarkers in the the pathogenesis, clinical manifestations, and therapeutic outcome of systemic sclerosis. Journal of Mosaic of Autoimmunity, pages 5, Apr 2025. URL: https://doi.org/10.53941/jmai.2025.100005, doi:10.53941/jmai.2025.100005. This article has 0 citations.
name: Systemic Sclerosis
creation_date: '2025-12-19T01:12:52Z'
updated_date: '2026-02-17T21:53:14Z'
category: Autoimmune
parents:
- Autoimmune Disease
- Connective Tissue Disease
disease_term:
preferred_term: Systemic Sclerosis
term:
id: MONDO:0005100
label: systemic sclerosis
description: >-
A chronic autoimmune connective tissue disease characterized by vasculopathy,
immune dysregulation, and progressive fibrosis of the skin and internal organs.
Classified into limited cutaneous and diffuse cutaneous subtypes based on
extent of skin involvement.
pathophysiology:
- name: Vascular Injury and Endothelial Dysfunction
description: >-
Early endothelial cell injury leads to vascular damage, intimal
proliferation, and obliterative vasculopathy. Raynaud's phenomenon
reflects vasospasm and structural vascular changes.
cell_types:
- preferred_term: Endothelial Cell
term:
id: CL:0000115
label: endothelial cell
biological_processes:
- preferred_term: Blood Vessel Development
term:
id: GO:0001568
label: blood vessel development
evidence:
- reference: PMID:38927538
reference_title: "Recent Insights into Cellular and Molecular Mechanisms of Defective Angiogenesis in Systemic Sclerosis."
supports: SUPPORT
snippet: >-
In systemic sclerosis (SSc, or scleroderma), defective angiogenesis, clinically
manifesting with abnormal capillary architecture and severe capillary reduction,
represents a hallmark of early-stage disease, usually preceding the onset of
tissue fibrosis, and is caused by several cellular and molecular mechanisms
affecting microvascular endothelial cells with different outcomes.
explanation: >-
This evidence supports the early vascular injury mechanism by demonstrating
that defective angiogenesis and capillary abnormalities precede fibrosis in
SSc.
- reference: PMID:38927538
reference_title: "Recent Insights into Cellular and Molecular Mechanisms of Defective Angiogenesis in Systemic Sclerosis."
supports: SUPPORT
snippet: >-
Indeed, once damaged, endothelial cells can be dysfunctionally activated, thus
becoming unable to undergo angiogenesis and promoting perivascular inflammation.
They can also undergo apoptosis, transdifferentiate into profibrotic myofibroblasts,
or acquire a senescence-associated secretory phenotype characterized by the
release
of exosomes and several profibrotic and proinflammatory mediators.
explanation: >-
This describes multiple mechanisms of endothelial dysfunction in SSc, including
impaired angiogenesis, endothelial-to-mesenchymal transition, and inflammatory
mediator release that contribute to vascular pathology.
- name: Immune Activation and Autoantibody Production
description: >-
Characteristic autoantibodies include anti-centromere (limited disease),
anti-Scl-70/topoisomerase I (diffuse disease), and anti-RNA polymerase III.
T cells and macrophages infiltrate affected tissues and produce
pro-fibrotic cytokines.
cell_types:
- preferred_term: CD4+ T Cell
term:
id: CL:0000624
label: CD4-positive, alpha-beta T cell
- preferred_term: Macrophage
term:
id: CL:0000235
label: macrophage
biological_processes:
- preferred_term: Immunoglobulin Production
term:
id: GO:0002377
label: immunoglobulin production
evidence:
- reference: PMID:38296975
reference_title: "GWAS for systemic sclerosis identifies six novel susceptibility loci including one in the Fcγ receptor region."
supports: PARTIAL
snippet: >-
Here we report the largest Asian genome-wide association study (GWAS) for
systemic sclerosis performed to date, based on data from Japanese subjects and
comprising of 1428 cases and 112,599 controls. The lead SNP is in the FCGR/FCRL
region, which shows a penetrating association in the Asian population, while
a
complete linkage disequilibrium SNP, rs10917688, is found in a cis-regulatory
element for IRF8.
explanation: >-
This genetic evidence implicates B cell and Fc receptor biology in SSc
susceptibility, with IRF8 being a key regulator of immune cell development
and interferon responses that contribute to immune dysregulation.
- reference: PMID:38296975
reference_title: "GWAS for systemic sclerosis identifies six novel susceptibility loci including one in the Fcγ receptor region."
supports: PARTIAL
snippet: >-
Prioritizing the top 5% of SNPs of IRF8 binding sites in B cells improves the
fitting of the polygenic risk scores, underscoring the roles of B cells and
IRF8 in the development of systemic sclerosis.
explanation: >-
This supports the role of B cell biology and autoantibody production in SSc
pathogenesis, demonstrating that B cell-specific genetic variants contribute
to disease risk.
- name: Fibroblast Activation and Fibrosis
description: >-
TGF-beta signaling drives fibroblast activation and differentiation into
myofibroblasts. Excessive collagen and extracellular matrix deposition
leads to progressive fibrosis of skin and internal organs.
cell_types:
- preferred_term: Fibroblast
term:
id: CL:0000057
label: fibroblast
- preferred_term: Myofibroblast
term:
id: CL:0000186
label: myofibroblast cell
biological_processes:
- preferred_term: Extracellular Matrix Organization
term:
id: GO:0030198
label: extracellular matrix organization
- preferred_term: TGF-beta Signaling
term:
id: GO:0007179
label: transforming growth factor beta receptor signaling pathway
evidence:
- reference: PMID:38147960
reference_title: "Fibroblast Subpopulations in Systemic Sclerosis: Functional Implications of Individual Subpopulations and Correlations with Clinical Features."
supports: PARTIAL
snippet: >-
SSc skin demonstrated an increased abundance of COMP+, COL11A1+, MYOC+, CCL19+,
SFRP4/SFRP2+, and PRSS23/SFRP2+ fibroblasts signatures and decreased proportions
of CXCL12+ and PI16+ fibroblast signatures in the Prospective Registry of Early
Systemic Sclerosis and Genetics versus Environment in Scleroderma Outcome Study
cohorts.
explanation: >-
This demonstrates the heterogeneity of fibroblast populations in SSc, with
increased profibrotic fibroblast subpopulations and decreased normal fibroblast
signatures contributing to excessive ECM deposition.
- reference: PMID:38147960
reference_title: "Fibroblast Subpopulations in Systemic Sclerosis: Functional Implications of Individual Subpopulations and Correlations with Clinical Features."
supports: PARTIAL
snippet: >-
The proportions of profibrotic COMP+, COL11A1+, SFRP4/SFRP2+, and PRSS23/SFRP2+
and proinflammatory CCL19+ fibroblast signatures were positively correlated
with
clinical and histopathological parameters of skin fibrosis, whereas signatures
of
CXCL12+ and PI16+ fibroblasts were inversely correlated.
explanation: >-
This provides direct evidence linking specific fibroblast subpopulations to
clinical severity of skin fibrosis, supporting the role of fibroblast activation
in SSc pathogenesis and disease progression.
phenotypes:
- name: Raynaud Phenomenon
category: Vascular
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Raynaud Phenomenon
term:
id: HP:0030880
label: Raynaud phenomenon
notes: Often the earliest manifestation
- name: Skin Thickening
category: Dermatological
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Thick Skin
term:
id: HP:0001072
label: Thickened skin
- name: Pulmonary Fibrosis
category: Respiratory
frequency: FREQUENT
phenotype_term:
preferred_term: Pulmonary Fibrosis
term:
id: HP:0002206
label: Pulmonary fibrosis
notes: Major cause of morbidity and mortality
- name: Dysphagia
category: Gastrointestinal
frequency: FREQUENT
phenotype_term:
preferred_term: Dysphagia
term:
id: HP:0002015
label: Dysphagia
notes: Due to esophageal dysmotility
biochemical:
- name: Anti-Scl-70 (Anti-Topoisomerase I)
presence: Elevated
context: Associated with diffuse cutaneous SSc and ILD
- name: Anti-Centromere Antibodies
presence: Elevated
context: Associated with limited cutaneous SSc
- name: Anti-RNA Polymerase III
presence: Elevated
context: Associated with rapidly progressive skin disease and renal crisis
genetic:
- name: HLA-DRB1
association: Risk Factor
- name: IRF5
association: Risk Factor
- name: STAT4
association: Risk Factor
treatments:
- name: Calcium Channel Blockers
description: For Raynaud's phenomenon management.
- name: Mycophenolate Mofetil
description: Immunosuppressant for skin and lung involvement.
- name: Nintedanib
description: Antifibrotic agent for progressive interstitial lung disease.
- name: ACE Inhibitors
description: Critical for scleroderma renal crisis management.
classifications:
harrisons_chapter:
- classification_value: musculoskeletal system disorder
- classification_value: connective tissue disease
- classification_value: autoimmune disease
references:
- reference: DOI:10.1007/s00296-024-05699-x
title: Heart involvement in patients with systemic sclerosis—what have we
learned about it in the last 5 years
findings: []
- reference: DOI:10.1007/s10238-022-00841-0
title: Transforming growth factor beta isoforms and TGF-βR1 and TGF-βR2
expression in systemic sclerosis patients
findings: []
- reference: DOI:10.1038/s41467-023-44541-z
title: GWAS for systemic sclerosis identifies six novel susceptibility loci
including one in the Fcγ receptor region
findings: []
- reference: DOI:10.1038/s41467-023-44645-6
title: Systems-based identification of the Hippo pathway for promoting
fibrotic mesenchymal differentiation in systemic sclerosis
findings: []
- reference: DOI:10.1136/rmdopen-2023-003148
title: Anti-topoisomerase, but not anti-centromere B cell responses in
systemic sclerosis display active, Ig-secreting cells associated with lung
fibrosis
findings: []
- reference: DOI:10.31138/mjr.270324.tis
title: 'Type I Interferons in Systemic Autoimmune Rheumatic Diseases: Pathogenesis,
Clinical Features and Treatment Options'
findings: []
- reference: DOI:10.3389/fimmu.2025.1551911
title: 'A review of recent studies on the pathogenesis of Systemic Sclerosis: focus
on fibrosis pathways'
findings: []
- reference: DOI:10.3389/fmolb.2023.1215039
title: 'Metabolic fingerprinting of systemic sclerosis: a systematic review'
findings: []
- reference: DOI:10.3390/biomedicines12061331
title: Recent Insights into Cellular and Molecular Mechanisms of Defective
Angiogenesis in Systemic Sclerosis
findings: []
- reference: DOI:10.3390/cimb45100490
title: 'Biomarkers in Systemic Sclerosis: An Overview'
findings: []
- reference: DOI:10.3390/ijms25094728
title: 'Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: From Bedside
to Bench and Back Again'
findings: []
- reference: DOI:10.3390/ijms26062421
title: 'The Role of CXCL4 in Systemic Sclerosis: DAMP, Auto-Antigen and Biomarker'
findings: []
- reference: DOI:10.53941/jmai.2025.100005
title: The Role of Biomarkers in the the Pathogenesis, Clinical
Manifestations, and Therapeutic Outcome of Systemic Sclerosis
findings: []
- reference: DOI:10.55563/clinexprheumatol/is29he
title: 'Systemic sclerosis: one year in review 2024'
findings: []