Esclerosis sistémica

Abstract

Systemic sclerosis, also known as scleroderma, is a systemic autoimmune disease characterised by fibrosing involvement of the skin and internal organs. It is a disease of unknown cause, and its aetiopathogenesis involves genetic and environmental factors and three fundamental processes: vascular involvement, immune system intervention and fibrosis. It predominates in females and peaks in middle age. Skin involvement is divided into two types according to extent: limited skin involvement and diffuse skin involvement. It is a very heterogeneous disease, with a wide spectrum of manifestations, which can present with mild and stable forms over time or rapidly progressive severe forms. Raynaud's phenomenon, digestive tract involvement, especially the oesophagus, pulmonary hypertension, interstitial pulmonary, musculoskeletal, and cardiac involvement are characteristic of the disease. Diagnosis of the disease is medical, based on symptoms, examination, and complementary tests. Some involvement may be asymptomatic on onset, and therefore early detection and treatment is important since many of its manifestations entail significant morbimortality.