title: [Therapeutic management of systemic sclerosis].
abstract: Improved understanding of the pathophysiology of systemic sclerosis (SSc) opens new therapeutic avenues in its treatment. The efficacy of disease-modifying agents remains limited however, and none has yet demonstrated its ability to improve survival in a prospective randomized trial.
  
  
  RESULTS
  of traditional antifibrotic agents such as colchicine and D-penicillamine are disappointing. Cyclophosphamide (CYC) seems to be beneficial in interstitial lung disease associated with SSc. Organ-specific therapies may produce dramatic benefits. Examples include angiotensin-converting enzyme inhibitors for renal failure and epoprostenol for primary pulmonary hypertension. Several new therapeutic approaches are currently under evaluation, including high-dose CYC followed by peripheral stem cell transplantation, vasodilators, and antiinflammatory and antifibrotic agents. Physical therapy and rehabilitation may help to treat disability and loss of function in SSc patients.
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, Assistance Publique-Hôpitaux de Paris, Université Paris-Descartes, Faculté de Médecine. luc.mouthon@cch.aphp.fr
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Alice
authors.initials: A
authors.lastname: Berezné
authors.forename: Serge
authors.initials: S
authors.lastname: Poiraudeau
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D058186:Acute Kidney Injury / Q000188:drug therapy / Q000209:etiology; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D003545:Cysteine / Q000008:administration & dosage / Q000031:analogs & derivatives / Q000627:therapeutic use; D011464:Epoprostenol / Q000008:administration & dosage / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D007372:Interferons / Q000008:administration & dosage / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000188:drug therapy / Q000209:etiology; D009989:Orthotic Devices; D010396:Penicillamine / Q000008:administration & dosage / Q000627:therapeutic use; D036102:Peripheral Blood Stem Cell Transplantation; D026741:Physical Therapy Modalities; D011446:Prospective Studies; D016032:Randomized Controlled Trials as Topic; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology / Q000534:rehabilitation / Q000628:therapy*; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000893:Anti-Inflammatory Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D000959:Antihypertensive Agents; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D003520:Cyclophosphamide; D007372:Interferons; D011464:Epoprostenol; D010396:Penicillamine; D003545:Cysteine; C026535:bucillamine