[Long-term treatments for systemic sclerosis: what are the perspectives?].
Systemic sclerosis (SSc) is responsible for fibrosis of the dermis and other organs as well as vascular abnormalities. While the pathogenesis of SSc is continually being better understood, there is still no single therapeutic agent that has been shown to increase survival in a prospective randomized trial. Traditional medications such as colchicine and D-penicillamine are disappointing in clinical practice, and the latter one failed to clearly show benefit when tested in a prospective placebo controlled trial comparing conventional high dose versus low dose. Conversely, new disease modifying agents are emerging such as cyclophosphamide (CYC) in interstitial pulmonary disease and stem cell autograft after high dose CYC therapy in patients who develop visceral involvement in the three first years of evolution of the disease. Organ specific therapy may show dramatic benefit, such as angiotensin converting enzyme inhibitors in renal crisis and epoprostenol in primary pulmonary hypertension. We will try to review disease modifying agents available in SSc and emphasize new therapeutic agents that are currently being evaluated, including vasodilators, anti-inflammatory, anti-fibrosing agents and immunosuppressive molecules.
Mouthon
Luc
L
Service de Médecine Interne, Hôpital Avicenne, Assistance Publique - Hôpitaux de Paris et Université Paris-XIII, 125, rue de Stalingrad, 93009 Bobigny Cedex, France. luc.mouthon@avc.ap-hop-paris.fr
Agard
Christian
C
Garcia De La Peña-Lefebvre
Paloma
P
Guillevin
Loïc
L
D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D013256:Steroids; D014665:Vasodilator Agents / Q000627:therapeutic use*
D000806:Angiotensin-Converting Enzyme Inhibitors; D000893:Anti-Inflammatory Agents; D000933:Antifibrinolytic Agents; D007166:Immunosuppressive Agents; D013256:Steroids; D014665:Vasodilator Agents