Management of interstitial lung disease in systemic sclerosis: lessons from SLS and FAST.
Interstitial lung disease (ILD) is a leading cause of death in systemic sclerosis (SSc). Two randomized controlled trials recently demonstrated the modest effects of cyclophosphamide on lung physiology (forced vital capacity) and extrapulmonary outcomes (dyspnea, function, quality of life, and skin thickening). Recommendations can now be made about the short-term management for SSc-ILD. However, many questions remain unanswered, including how long to treat with cyclophosphamide; whether patients should take maintenance therapy after the initial or induction phase; whether there are alternative therapies; how to treat patients with ILD and pulmonary hypertension; and how to treat patients with severe ILD.
Fathi
Nihal
N
David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA.
Furst
Daniel E
DE
Clements
Philip J
PJ
D003520:Cyclophosphamide / Q000627:therapeutic use; D004185:Disability Evaluation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008168:Lung / Q000187:drug effects / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D016032:Randomized Controlled Trials as Topic; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide