Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) is a chronic, autoimmune disease which can affect the blood vessels, the visceral organs, and the skin. SSc, most commonly, develops between the ages of 30 and 50, but it can be seen at any age. In terms of skin involvement, SSc can be classified as limited or diffuse. Its etiopathogenesis is still unclear. Microvascular dysfunction is thought to be followed by immunological activation, collagen and extracellular matrix deposition, and finally fibrosis. Diagnosis is based on clinical presentation. Sclerosis of the metacarpophalangeal and/or metatarsophalangeal joints is the major diagnostic criterion, whereas sclerodactylia, digital ulcers (DU), and pulmonary fibrosis are the minor criteria. SSc is diagnosed with one major criterion or two minor criteria. Detection of autoantibodies can help the diagnosis. Antinuclear antibody (ANA), anti-centromere antibody, anti-scl 70, RNA polymerase 1 and 3, and anti-fibrillin antibody can be found positive in SSc. SSc must be differentiated from all sclerosing diseases and the diseases with Raynaud’s phenomenon. Visceral diseases, such as primary pulmonary hypertension, primary biliary cirrhosis, and infiltrative cardiomyopathy, should also be considered in its differential diagnosis. The main treatment goal is to target visceral involvement.