SYSTEMIC SCLEROSIS (SCLERODERMA) COMPREHENSIVE ANALYSIS OF PATHOPHYSIOLOGY, CLINICAL SUBTYPES, DIAGNOSTIC ADVANCES, AND THERAPEUTIC HORIZONS
Keywords:
Keywords: Systemic Sclerosis, Scleroderma, Fibrosis, Vasculopathy, Interstitial Lung Disease, Anti-Scl-70, Nintedanib.Abstract
Abstract
Systemic sclerosis (SSc, commonly known as scleroderma) is a heterogeneous, multisystem
autoimmune disease characterized by a triad of severe vascular dysfunction, immune dysregulation with
specific autoantibody production, and widespread progressive tissue fibrosis. SSc exhibits the highest
individual case fatality rate among all systemic autoimmune rheumatic diseases. This review provides
a comprehensive synthesis of current scientific understanding regarding SSc. We explore the
pathophysiological signaling cascades involving transforming growth factor-beta (TGF-β), endothelin-1 (ET-1), and vascular endothelial growth factor (VEGF).
References
References
1. Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nat Rev Dis Primers.
2015;1:15002.
2. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699.
3. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for Systemic SclerosisAssociated Interstitial Lung Disease. N Engl J Med. 2019;380(26):2518-2528.
4. Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised,
double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8(10):963-974.
5. Van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic
sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative
initiative. Ann Rheum Dis. 2013;72(11):1747-1755.
6. Volkmann ER, Tashkin DP, Sim M, et al. Short- and long-term outcomes of
immunosuppressive therapy in systemic sclerosis-associated interstitial lung disease. Arthritis
Rheumatol. 2022;74(7):1210-1219.