Autoimmunity in systemic sclerosis

Current concepts

Francesco Boin, Antony Rosen

Research output: Contribution to journalArticle

Abstract

Systemic sclerosis (SSc) is characterized by tissue fibrosis, obliterative microangiopathy, and immune abnormalities. The role of autoimmunity in generating the clinical and pathologic phenotype in SSc remains uncertain. Distinct subsets of antinuclear antibodies are selectively associated with unique disease manifestations but do not have a proven pathogenic role. A new class of autoantibodies recognizing cellular or extracellular matrix antigens has been recognized in SSc patients. They seem to directly activate pathways that may contribute to SSc-specific tissue and vascular damage. Data confirms that activation and polarization of T cells can contribute to a profibrotic environment. Also, activated immune effector cells can promote vascular obliterative damage through direct cytotoxic pathways targeting the endothelium or by inducing proinflammatory molecules. Technologies are emerging to accurately measure the autoantigen-specific T-cell response in SSc patients. Perturbed B-cell homeostasis has been reported in SSc. If confirmed in-vivo, these advances could lead to new disease-modifying therapeutic strategies directed at SSc-specific immune effector pathways.

Original languageEnglish (US)
Pages (from-to)165-172
Number of pages8
JournalCurrent Rheumatology Reports
Volume9
Issue number2
DOIs
StatePublished - May 2007

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Systemic Scleroderma
Autoimmunity
Blood Vessels
T-Lymphocytes
Antinuclear Antibodies
Autoantigens
Autoantibodies
Endothelium
Extracellular Matrix
Homeostasis
Fibrosis
B-Lymphocytes
Technology
Phenotype
Antigens

ASJC Scopus subject areas

  • Rheumatology

Cite this

Autoimmunity in systemic sclerosis : Current concepts. / Boin, Francesco; Rosen, Antony.

In: Current Rheumatology Reports, Vol. 9, No. 2, 05.2007, p. 165-172.

Research output: Contribution to journalArticle

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