Although scleroderma-associated interstitial lung disease (SSc-ILD) is a significant contributor to both morbidity and mortality, its pathogenesis is largely unclear. Pulmonary function tests and high-resolution computed tomographic scanning continue to be the most effective tools to screen for lung involvement and to monitor for disease progression. More research and better biomarkers are needed to identify patients most at risk for developing SSc-ILD as well as to recognize which of these patients will progress to more severe disease. Although immunosuppression remains the mainstay of treatment, antifibrotic agents may offer new avenues of treatment for patients with SSc-ILD in the future.
Interstitial lung disease (ILD) is the leading cause of mortality for patients with SSc.
Early diagnosis is critical for managing patients with scleroderma-associated interstitial lung disease (SSc-ILD).
Pulmonary function tests (PFTs) and high-resolution computed tomographic (HRCT) chest imaging are used for screening and management.
Immunosuppression can be beneficial, but better therapies are needed.