Current perspectives on the immunopathogenesis of systemic sclerosis
Open Access
- 1 April 2016
- journal article
- review article
- Published by Taylor & Francis Ltd in ImmunoTargets and Therapy
- Vol. ume 5, 21-35
- https://doi.org/10.2147/itt.s82037
Abstract
Current perspectives on the immunopathogenesis of systemic sclerosis Patrizia Fuschiotti Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA Systemic sclerosis (SSc or scleroderma) is a progressive and highly debilitating autoimmune disorder characterized by inflammation, vasculopathy, and extensive fibrosis. SSc is highly heterogeneous in its clinical presentation, extent and severity of skin and internal organ involvement, and clinical course and has the highest fatality rate among connective tissue diseases. While clinical outcomes have improved in recent years, no current therapy is able to reverse or slow the natural progression of SSc, a reflection of its complex pathogenesis. Although activation of the immune system has long been recognized, the mechanisms responsible for the initiation of autoimmunity and the role of immune effector pathways in the pathogenesis of SSc remain incompletely understood. This review summarizes recent progress in disease pathogenesis with particular focus on the immunopathogenetic mechanisms of SSc. Keywords: scleroderma, immune mediators, inflammation, autoimmunityKeywords
This publication has 98 references indexed in Scilit:
- Polymorphisms in TBX21 and STAT4 increase the risk of systemic sclerosis: Evidence of possible gene–gene interaction and alterations in Th1/Th2 cytokinesArthritis & Rheumatism, 2009
- Association of TNFSF4 (OX40L) polymorphisms with susceptibility to systemic sclerosisAnnals Of The Rheumatic Diseases, 2009
- Human Parvovirus B19 (B19V) Infection in Systemic Sclerosis PatientsIntervirology, 2009
- Course of the modified Rodnan skin thickness score in systemic sclerosis clinical trials: Analysis of three large multicenter, double‐blind, randomized controlled trialsArthritis & Rheumatism, 2009
- Major histocompatibility complex (MHC) class II alleles, haplotypes and epitopes which confer susceptibility or protection in systemic sclerosis: analyses in 1300 Caucasian, African-American and Hispanic cases and 1000 controlsAnnals Of The Rheumatic Diseases, 2009
- An Essential Role for Fibronectin Extra Type III Domain A in Pulmonary FibrosisAmerican Journal of Respiratory and Critical Care Medicine, 2008
- Effects of 1-Year Treatment with Cyclophosphamide on Outcomes at 2 Years in Scleroderma Lung DiseaseAmerican Journal of Respiratory and Critical Care Medicine, 2007
- Systemic sclerosis: a prototypic multisystem fibrotic disorderJCI Insight, 2007
- Transcription factor T-bet regulates skin sclerosis through its function in innate immunity and via IL-13Proceedings of the National Academy of Sciences of the United States of America, 2007
- BAFF: A fundamental survival factor for B cellsNature Reviews Immunology, 2002