Management of systemic sclerosis-associated interstitial lung disease

To review the recently published data and provide a practical overview for management of systemic sclerosis-interstitial lung disease (SSc-ILD).

Published evidence shows considerable practitioner variability in screening patients for ILD. Recent published data support use of cyclophosphamide or mycophenolate mofetil as first-line treatment of SSc-ILD. For patients not responding to first-line therapies, consideration is given to rituximab as rescue therapy. Recent trials of hematopoietic autologous stem cell transplantation have demonstrated benefit in patients with progressive SSc-ILD. Antifibrotic agents are approved in idiopathic pulmonary fibrosis; studies with antifibrotics are underway for SSc-ILD.

The specter of rapidly progressive lung disease requires clinicians to risk stratify patients according to known predictors for progression and rigorously monitor for symptoms and advancing disease. The abovementioned therapies promise improved efficacy and favorable side-effect profiles compared to cyclophosphamide.