CLINICAL, GENETIC AND PROGNOSTIC FACTORS IN SYSTEMIC SCLERODERMA

Abstract

Systemic sclerosis, a chronic autoimmune connective tissue disease, is marked by skin thickening, Raynaud's phenomenon, visceral organ damage, and musculoskeletal involvement, presenting a significant challenge in rheumatology and immunology [7,8]. The pathogenesis of systemic sclerosis involves intricate interactions between the immune system, vasculature, and connective tissue, necessitating simultaneous dysfunction in immune tolerance, endothelial physiology, and extracellular matrix turnover [1,5]. The varied clinical presentation of systemic sclerosis, ranging from limited cutaneous involvement to diffuse systemic manifestations, underscores the disease's heterogeneous nature and the need for personalized treatment strategies [2,9]. Despite the disease's relatively low incidence, approximately 13 per 1 million people per year, and a prevalence of about 200 per 1 million, its impact on patient morbidity and mortality is substantial, necessitating ongoing research into its underlying mechanisms and therapeutic interventions [10,12]. The disease's complexity is further compounded by the diverse array of autoantibodies associated with systemic sclerosis, including anti-centromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies, which correlate with distinct clinical phenotypes and disease outcomes.