Progressive Interstitial Lung Disease Non-Responsive to Immunosuppressive Therapy

Immunosuppressive therapy with mycophenolate mofetil or cyclophosphamide is generally considered to be first-line therapy for scleroderma-associated interstitial lung disease (SSc-ILD). Not all patients are responsive to or are able to tolerate immunosuppressive therapy, indicating the need for additional therapeutic options. We present such a patient whose lung disease failed to respond to immunosuppressive therapy, and we discuss other potential therapeutic options, including biologic agents and anti-fibrotic medications.