Abstract

Systemic sclerosis (scleroderma) has a complex pathogenesis. Thus, management of systemic sclerosis remains a challenge. Currently, treatment in systemic sclerosis is targeted at reducing 1) abnormal immune activation, 2) microvascular abnormalities and 3) fibrosis. Therapy should be adjusted individually, depending on disease subtype and activity. Immunosuppressive agents, such as cyclophosphamide or mycophenolate mofetil and occasionally methotrexate or cyclosporine A are indicated in rapidly progressing systemic sclerosis. Low or moderate doses of corticosteroids may be used in patients with musculoskeletal involvement. Reports of effective biological therapy with rituximab or infliximab are available. In cases of rapidly progressing systemic sclerosis high dose immunosuppressive or cytostatic agents with subsequent autologous bone marrow transplantation may be indicated. Novel therapies have the potential of significantly modifying disease process and improving overall clinical outcome in patients with systemic sclerosis.