Systemic Sclerosis-Related Interstitial Lung Disease

Systemic sclerosis is a multisystem autoimmune disease with potential lung manifestations that include pulmonary vascular and interstitial disease. Not only is interstitial lung disease the most common pulmonary manifestation, it is a major cause of morbidity and mortality in patients with systemic sclerosis. The risk of systemic sclerosis patients developing progressive interstitial lung disease is strongly associated with the presence of certain autoantibodies The most common lung injury pattern observed in systemic sclerosis-related interstitial lung disease is the fibrotic variant of nonspecific interstitial pneumonia. Because scleroderma renal crisis can now be effectively treated, recently, focus has turned toward how best to treat systemic sclerosis-related interstitial lung disease. Therapy usually involves an immunomodulatory regimen consisting of a steroid-sparing agent with or without glucocorticoids, and response to therapy is variable.