[Contemporaneous clinical picture of systemic sclerosis]

Systemic sclerosis is a multi-system disease characterized by skin fibrosis and visceral involvement. The course of disease is unpredictable, sometimes stable for years sometimes rapidly progressive, leading to death during months. Prognosis is limited by internal organ involvement. Nowadays leading cause of death is interstitial lung fibrosis and pulmonary arterial hypertension. In this paper we present two subsets of systemic sclerosis: limited and diffuse and newly proposed classification for early systemic sclerosis, including objective documentation of Raynaud's phenomenon and systemic sclerosis-type naifold capillary pattern or presence of selective auto-antibodies (anti-centromer or anti-Scl 70). We describe clinical symptoms, internal organ involvement and tools to its detection, with use of HRCT, Doppler echocardiography, spirometric gas transfer (DLCO). We present current approach to staging the disease according to parameters proposed by Medsger et al. involving general symptoms, Raynaud's phenomenon severity, Rodnan skin score, musculoskeletal symptoms, lung, heart and kidney involvement. Factors related to scleroderma renal crisis are presented, to provide clinical evaluation of patients at risk of this complication.