Systemic Sclerosis - An Update

Systemic sclerosis (SSc) is a clinically heterogeneous, systemic disorder affecting connective tissue of skin, internal organs, and walls of blood vessels. It is characterized by alterations of the microvasculature in the form of hypoxia, digital ulcers, and pulmonary arterial hypertension; disturbances of the immune system, including dysbalance of cytokine expression, autoantibodies (Auto-ab), and abnormalities of blood progenitor and/or effector cells; and by massive deposition of collagen in the connective tissue of the skin and various internal organs. This review discusses epidemiology and survival; clinical features including subsets and internal organ involvement; pathophysiology including genetics, microvasculature, immunobiology, fibroblasts (FBs), and connective tissue metabolism; and environmental factors. Early diagnosis and individually tailored therapy help to manage this disorder. Therapy involves immunomodulation and targeting of blood vessels and fibrosis. The multicenter online database "European Scleroderma Trials and Research" project allows further insight of prognostic factors and conception of new therapies. Physical and psychotherapy are important.