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
