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Semin Respir Crit Care Med 2019; 40(02): 271-277
DOI: 10.1055/s-0039-1684007
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Interstitial Pneumonia with Autoimmune Features and Undifferentiated Connective Tissue Disease

Andrea K. Chernau
1   Rocky Vista University College of Osteopathic Medicine, Parker, Colorado
,
Paolo M. Leone
2   Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy
3   Università Cattolica del Sacro Cuore, Roma, Italy
4   Interstitial Lung Disease Program, National Jewish Health, Denver, Colorado
,
Jeffrey J. Swigris
4   Interstitial Lung Disease Program, National Jewish Health, Denver, Colorado
› Author Affiliations
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Publication History

Publication Date:
28 May 2019 (online)

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Abstract

In 2015, a multidisciplinary task force comprising pulmonologists, rheumatologists, pathologists, and radiologists representing the European Respiratory Society and American Thoracic Society published a diagnostic classification schema for individuals with interstitial lung disease and autoimmune features who did not meet criteria for a defined connective tissue disease. The term interstitial pneumonia with autoimmune features (IPAF) was applied. Classification criteria are often nonspecific, but up to 90% of subjects with IPAF have serological evidence for autoimmunity (particularly (+) antinuclear antibodies). Distinguishing patients with IPAF from idiopathic pulmonary disorders may be difficult. The natural history and appropriate management of IPAF have not been clarified, as data are largely limited to retrospective studies. In this review, we discuss the salient clinical, serologic, histologic, and radiographic features of IPAF and discuss an approach to management.

Keywords

interstitial lung disease - autoimmune disease - connective tissue disease - interstitial pneumonia with autoimmune features (IPAF) - antinuclear antibody