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Clin Chest Med. 2019 Sep;40(3):609-616. doi: 10.1016/j.ccm.2019.05.007.

Interstitial Pneumonia with Autoimmune Features.

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Divisions of Rheumatology, Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus, University of Colorado School of Medicine, 12631 East 17th Avenue, Academic Office Building One, Aurora, CO 80045, USA. Electronic address:


The European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease put forth the research classification interstitial pneumonia with autoimmune features as a step toward uniformly describing these patients. Diverse nomenclature and classification schemes had been proposed to characterize them. This classification has provided uniform nomenclature and criteria, fostering interdisciplinary engagement and research. Longitudinal surveillance is needed; some patients evolve to a defined connective tissue disease. This review discusses cohort studies of interstitial pneumonia with autoimmune features and what they have taught us about the phenotype, and offers insights into future directions.


Connective tissue diseases; Idiopathic interstitial pneumonias; Interstitial lung disease; Pulmonary fibrosis


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