Rheumatological Conditions and the Kidney

Abstract

Systemic sclerosis (SSc) is a debilitating, chronic, systemic, autoimmune disease of unknown cause. It is classified as limited or diffuse dependent on skin involvement. The incidence and prevalence of SSc vary in different populations where it seems to be more prevalent in the United States (286 cases per million adults) than in Europe (31 cases per million adults) with an annual incidence of 1–20 cases per million. SSc is three times more common in females than males and typically presents between the ages of 30 and 60 years. Typical skin features of SSc are well described and include skin thickening, sclerodactyly, finger pulp pitting, fingertip ulceration, digital gangrene and telangiectasia; some of which are illustrated in Figs. 27.1 and 27.2. SSc causes vascular damage, immune activation and inflammation, culminating in fibrosis which is responsible for the clinical manifestations of the disease. Table 27.1 summarizes the prevalence of extrarenal clinical manifestations seen in SSc.