Lung disease is a common manifestation of connective tissue disease (CTD) and is associated with significant morbidity and mortality. The evaluation of lung disease, and interstitial lung disease (ILD) in particular, in patients with CTD is complex because of the heterogeneity of the CTDs, the varied types and degrees of severity of ILD encountered, and because ILD can be identified at any point in time in these patients.

Cross-disciplinary, thorough evaluations are needed when CTD patients develop ILD or when evaluating ILD patients for the presence of occult CTD. Determining that ILD is associated with an established CTD requires the exclusion of alternative etiologies, and thorough assessments of the clinical features of both the CTD and I...