Cited by 119Systemic Sclerosis and Related DiseasesPulmonary Hypertension Research and TreatmentsConnective tissue disorders research

Vascular involvement in systemic sclerosis (scleroderma)

Open access
Postlethwaite, Pattanaik, Debendra, Brown, Monica·2011·Journal of Inflammation Research

Abstract

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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Crossref · 10.2147/jir.s18145

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249 of 249 references verified0 citation errors · 0 not checkable

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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].”
  • 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].”

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1 retracted

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Authors
Postlethwaite, Pattanaik, D., & Brown, M.
Affiliations
University of Tennessee Health Science Center (US)
Venue
Journal of Inflammation Research
Published
2011-07
Volume
4
Pages
105
ISSN
1178-7031 (ISSN-L)
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
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

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