[Systemic sclerosis. Which therapies?].

Abstract

Sistemic sclerosis is a connective tissue disease characterized by excessive deposit of collagen in the skin and in different viscera such as the lung, the heart, the kidney, the gastrointestinal tract and muscoloskeletal system. Fibrosis is a natural and not reversible consequence of this extracellular matrix accumulation. Past management strategies have not been successful, usually. New physiopathologic knowledges establish the bases to obtain a more successful control of the disease evolution. Literature data underline the importance of a timely and personalized therapy based on kind, seriousness and stage of internal organs involvement to allow the patient to continue normal public relations, a good quality of life and the improvement of its global prognosis of this disease, often cause of disability and death. Actually, drug therapeutic management is based on combined use of vascular drugs: topical (Nitroglycerin patches), oral (Ace inhibitors and Calcium channel blockers) or, in severe cases, parenteral vasodilators (Prostaglandins and Prostacyclins), immunosuppressive therapies (Cyclophosphamide, Cyclosporin A, Methotrexate) and antifibrotic drugs (Penicil-lamine, Interferons).