Pathogenesis of systemic sclerosis—current concept and emerging treatments

Masutaka Furue, Chikage Mitoma, Hiroki Mitoma, Gaku Tsuji, Takahito Chiba, Takeshi Nakahara, Uchi Hiroshi, Takafumi Kadono

研究成果: Contribution to journalReview article査読

30 被引用数 (Scopus)

抄録

Systemic sclerosis (SSc) is an intractable multifaceted disease with high mortality. Although its pathogenesis is not fully understood, recent studies have advanced our knowledge on SSc. The cardinal pathological features of SSc are autoimmunity, vasculopathy, and fibrosis. The B cells in SSc are constitutively activated and lead to the production of a plethora of autoantibodies, such as anti-topoisomerase I and anti-centromere antibodies. In addition to these autoantibodies, which are valuable for diagnostic criteria or biomarkers, many other autoantibodies targeting endothelial cells, including endothelin type A receptor and angiotensin II type I receptor, are known to be functional and induce activation or apoptosis of endothelial cells. The autoantibody-mediated endothelial cell perturbation facilitates inflammatory cell infiltration, cytokine production, and myofibroblastic transformation of fibroblasts and endothelial cells. Profibrotic cytokines, such as transforming growth factor β, connective tissue growth factor, interleukin 4/interleukin 13, and interleukin 6, play a pivotal role in collagen production from myofibroblasts. Specific treatments targeting these causative molecules may improve the clinical outcomes of patients with SSc. In this review, we summarize recent topics on the pathogenesis (autoantibodies, vasculopathy, and fibrosis), animal models, and emerging treatments for SSc.

本文言語英語
ページ(範囲)790-797
ページ数8
ジャーナルImmunologic Research
65
4
DOI
出版ステータス出版済み - 8 1 2017

All Science Journal Classification (ASJC) codes

  • Immunology

フィンガープリント 「Pathogenesis of systemic sclerosis—current concept and emerging treatments」の研究トピックを掘り下げます。これらがまとまってユニークなフィンガープリントを構成します。

引用スタイル