Scleroderma lung: pathogenesis, evaluation, and current therapy.

Abstract

Interstitial lung disease (ILD) is now the most common cause of mortality in scleroderma, although its pathogenesis remains poorly understood. Management requires early detection and treatment before the onset of lung fibrosis. In a number of uncontrolled studies, the combination of daily oral cyclophosphamide and low-dose prednisone appears to be effective, although these conclusions have yet to be confirmed.