Connective Tissue Disease-Associated Interstitial Lung Disease

Abstract

Interstitial lung disease (ILD) is the most common pulmonary manifestation of connective tissue disease (CTD). It may develop in the patients with preexisting connective tissue diseases or may be the initial lone manifestation of an underlying CTD. Since ILD causes a potentially substantial morbidity and mortality in the patients with CTD, close collaboration among pulmonologists, rheumatologists, radiologists and pathologists is very essential to optimize the diagnostic evaluation and treatment. Important information on the pattern and extent of the ILD can be obtained by chest HRCT imaging. In general, surgical lung biopsy is not recommended for CTD-associated ILD. All patients with CTD-associated ILD do not require pharmacologic treatment. The decision to treat CTD-associated ILD often depends on 1) whether the patient is clinically impaired by the ILD, 2) whether the ILD is progressive, and 3) what contraindications exist. Differential diagnosis from superimposed pneumonia and drug-induced ILD is quite important. Immunosuppressive therapy is the mainstay of treatment. Recent success of cyclophosphamide in the clinical trials of systemic sclerosis-associated ILD is promising. (Korean J Med 2013;84:498-501)