Interstitial Lung Disease Developing during Treatment with Cyclosporine in a patient with Diffuse Scleroderma,...

Systemic sclerosis (scleroderma) is an uncommon disease of as-yet-unknown etiology. The hallmark of scleroderma is proliferation of collagen, particularly types I and III, with associated vascular changes. Visceral involvement, particularly pulmonary interstitial disease, can lead to significant morbidity and mortality.Specific therapy is unavailable to date, but there has been interest in the use of cyclosporine (CSA), because it inhibits interleukin-2 production and affects cytotoxic T cells. We present a case of a patient with a triad of suspected autoimmune diseases, systemic sclerosis and Crohn's. disease on CSA therapy for aplastic anemia. Despite hematologic response and cutaneous improvement on CSA therapy, this patient developed interstitial lung disease consistent with sclerodermatous lung.