Esclerosis sistémica

Abstract

Systemic sclerosis (SSc) is a complex systemic autoimmune disease characterized by progressive fibrosis of the skin and internal organs. Its etiology remains unknown, although its pathogenesis involves a multifactorial interaction between genetic predisposition, environmental factors, and three key processes: vascular damage, immune dysfunction, and fibrosis. It is more common in women, especially in middle age, and is classified into two main subtypes according to the extent of cutaneous involvement: limited and diffuse. It is a remarkably heterogeneous disease, with a clinical spectrum ranging from mild, stable forms to severe presentations with rapid progression. Among its most characteristic manifestations are Raynaud's phenomenon, gastrointestinal involvement (especially esophageal), pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), musculoskeletal abnormalities, and cardiac complications.

This review will delve into the main clinical manifestations of SSc, current diagnostic criteria, and recent advances in its therapeutic management.