Management of connective tissue disease-associated interstitial lung disease.

A thorough, often multidisciplinary assessment to determine extrathoracic versus intrathoracic disease activity and degrees of impairment is needed to optimize the management of connective tissue disease (CTD)-associated interstitial lung disease (ILD). Pharmacologic intervention with immunosuppression is the mainstay of therapy for all forms of CTD-ILD, but should be reserved for those that show clinically significant and/or progressive disease. The management of CTD-ILD is not yet evidence based and there is a need for controlled trials across the spectrum of CTD-ILD. Nonpharmacologic management strategies and addressing comorbidities or aggravating factors should be included in the comprehensive treatment plan for CTD-ILD.