The Spectrum of Interstitial Lung Disease in Connective Tissue Disease

Interstitial lung disease (ILD) is a common cause of morbidity and mortality in patients with connective tissue disease (CTD). In a minority of patients the ILD may be the presenting (or only) manifestation of an underlying CTD. Diagnosis of CTD-related ILD relies on a multidisciplinary team including pulmonologists, pathologists, radiologists, and rheumatologists, as the imaging and pathologic findings may be indistinguishable from idiopathic interstitial pneumonias. Moreover, many patients with ILD are suspected of having an underlying CTD but do not meet all of the necessary criteria for a specific disorder. This article provides a pattern-based approach to the imaging of CTD-related ILD and also reviews relevant clinical, pathologic, and serologic data that radiologists should be familiar with as part of a multidisciplinary team.