Interstitial lung disease (ILD) is a frequent complication of patients with connective tissue disease (CTD) and significantly affects morbidity and mortality. Disease course may vary from stable or mildly progressive to more severe, with rapid loss of lung function. We conducted a search of PubMed (National Library of Medicine) and the Web of Science Core Collection using the key words lung, pulmonary, pneumonia, pneumonitis, and alveolar and subtypes of CTD. All clinical studies from January 1, 1980, through September 1, 2018, were reviewed for descriptions of specific therapies and their efficacy or safety and were categorized as controlled interventional trials, observational prospective or retrospective cohort studies, case series (>5 patients), and case reports (<5 patients). Low-quality reports (<5 patients) before 2000, reviews, editorials, popular science papers, and letters to the editor without complete descriptions of the therapies used or their outcomes were excluded. Directed therapy for CTD-ILD is dominated by empirical use of immunosuppressive agents, with the decision to treat, treatment choice, and treatment duration limited to cases and cohort observations. Only a few higher-level controlled studies were available specifically in scleroderma-related ILD. We summarize herein for the clinician the published treatment scope and experience, highlighted clinical response, and common adverse reactions for the management of CTD-ILD.
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