Diagnosis and Management of Patients with Connective Tissue Disease-related Fibrosing Interstitial Lung Diseases

Fibrotic interstitial lung disease is an important driver of morbidity and mortality in patients with connective tissue diseases (CTD). Due to the lack of prospective randomized trial data in this population, practice pattern variation exists in the management of patients with CTD.

This case series describes three patients, each with a different background of autoimmunity complicated by fibrotic interstitial lung disease (ILD). We review their initial presentations, follow their disease trajectories on currently available treatments, and reference forthcoming clinical trials.

Clinical impact or potential implications. Response to immunosuppression and antifibrotic therapy is variable in patients with connective tissue disease-related fibrosing interstitial lung disease. Data from prospective clinical trials and longitudinal registry studies will conceivably provide additional insight into improving care for these patients.