Vascular involvement in systemic sclerosis (scleroderma)
Open accessAbstract
Systemic sclerosis (SSc) is an acquired multiorgan connective tissue disease with variable mortality and morbidity dictated by clinical subset type. The etiology of the basic disease and pathogenesis of the systemic autoimmunity, fibrosis, and fibroproliferative vasculopathy are unknown and debated. In this review, the spectrum of vascular abnormalities and the options currently available to treat the vascular manifestations of SSc are discussed. Also discussed is how the hallmark pathologies (ie, how autoimmunity, vasculopathy, and fibrosis of the disease) might be effected and interconnected with modulatory input from lysophospholipids, sphingosine 1-phosphate, and lysophosphatidic acid.
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Endothelial damage in systemic sclerosis contributes to platelet aggregation and release of fibrogenic mediators such as TGFβ1, TGFβ2, IL-4, PDGF, connective tissue factor, sphingosine 1-phosphate, and lysophosphatidic acid.
“Endothelial damage contributes to ongoing platelet aggregation with release of a member of fibrogenic mediators such as TGFβ1, TGFβ2, IL-4, platelet derived growth factor (PDGF), connective tissue factor, sphingosine 1-phosphate and lysophosphatidic acid [252].”
From Steroidogenesis in the skin: Implications for local immune functions (2013) · cites this paper as [252]
In systemic sclerosis, Th17 and Th2 predominance with IL-17, IL-4, and IL-13 release at lymphocytic infiltration sites contributes to fibrosis.
“There is also predominance of Th17 and Th2 cells with release of IL-17, IL-4 and IL-13 at sites of lymphocytic infiltration which contributes to ongoing fibrosis [252].”
From Steroidogenesis in the skin: Implications for local immune functions (2013) · cites this paper as [252]
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RetractedA novel potent vasoconstrictor peptide produced by vascular endothelial cells
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THE IMPACT OF ANTI B-CELL THERAPY ON ARTERIAL STIFFNESS IN SYSTEMIC SCLERODERMA
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- Authors
- Postlethwaite, Pattanaik, D., & Brown, M.
- Affiliations
- University of Tennessee Health Science Center (US)
- ORCID
- Pattanaik, Debendra
- Venue
- Journal of Inflammation Research
- Publisher
- Informa UK Limited
- Published
- 2011-07
- Volume
- 4
- Pages
- 105
- ISSN
- 1178-7031 (ISSN-L)
- PMC
- PMC3218751
- OpenAlex
- W2156270541
- Cited by
- 119
- References
- 251
- Citations by year
- 2026: 1 · 2025: 11 · 2024: 7 · 2023: 5 · 2022: 11 · 2021: 6 · 2020: 6 · 2019: 9 · 2018: 10 · 2017: 9 · 2016: 8 · 2015: 16 · 2014: 5 · 2013: 12 · 2012: 3
- FWCI
- 4.50
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Journal of Inflammation Research (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Dove Medical Press (Taylor and Francis Group) (submittedVersion, cc-by-nc)
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Keywords
- systemic sclerosis, vascular involvement, vasculopathy, autoimmunity, fibrosis, lysophospholipids, sphingosine-1-phosphate, lysophosphatidic acid
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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