Systemic sclerosis (scleroderma)

Abstract

Systemic sclerosis is a rare and often debilitating disorder characterized by a pathological triad: increased deposition of extracellular matrix and collagen in tissues, microvascular damage and dysfunction, and immune activation as evidenced by inflammation and frequent occurrence of autoantibodies. Until recently, therapeutic interventions were disappointing, given their inability to alter the natural course of this disease. The recognition that early detection and treatment of visceral complications are essential for stabilising, or in some cases, reversing their progression, has contributed greatly to improving the prognosis of systemic sclerosis. Furthermore, an increased interest in understanding the pathogenic mechanisms of this complex disease has opened new perspectives for therapy, targeting the diverse and inter-related components of the characteristic triad.