[Treatment of scleroderma].
No single therapeutic agent has been shown to increase survival of patients with systemic scleroderma in a prospective randomized trial. Drugs as colchicine and D-penicillamine are disappointing. Conversely, disease modifying agents are emerging such as cyclophosphamide in interstitial pulmonary disease, and stem cell autograft after high dose cyclophosphamide therapy in patients who develop visceral involvement in the three first years of evolution of the disease is under study. Organ specific therapy may show significant benefit, such as angiotensin converting enzyme inhibitors in renal crisis and epoprostenol in primary pulmonary hypertension.
Mouthon
Luc
L
Service de médecine interne Hôpital Avicenne (AP-HP), Université Paris XIII 93009 Bobigny. luc.mouthon@avc.ap-hop-paris.fr
D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D003520:Cyclophosphamide / Q000627:therapeutic use*; D011464:Epoprostenol / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D051437:Renal Insufficiency / Q000188:drug therapy / Q000209:etiology; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D014665:Vasodilator Agents / Q000627:therapeutic use*
D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D003520:Cyclophosphamide; D011464:Epoprostenol