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. 2017 Sep;24(5):191-196.
doi: 10.1097/CPM.0000000000000227.

Interstitial Pneumonia with Autoimmune Features: Overview of proposed criteria and recent cohort characterization

Cathryn T Lee  1 , Justin M Oldham  2
Affiliations

Interstitial Pneumonia with Autoimmune Features: Overview of proposed criteria and recent cohort characterization

Cathryn T Lee et al. Clin Pulm Med. 2017 Sep.

Abstract

The accurate diagnosis of interstitial lung disease (ILD) is essential for optimal prognostication and management. While connective tissue disease (CTD) is among the most common causes of ILD, some patients have features suggestive of autoimmunity without meeting criteria for a specific CTD. To help define and study this disease entity more uniformly, a 2015 research statement proposed consensus-based criteria and coined the term "interstitial pneumonia with autoimmune features" (IPAF). In this review, we summarize and compare previously proposed criteria to characterize these patients, provide an overview of the IPAF criteria and highlight recent investigations aimed at characterizing IPAF cohorts. We then call attention to questions that have arisen with the application of the IPAF criteria and discuss future areas of study.

Keywords: connective tissue disease (CTD); idiopathic pulmonary fibrosis (IPF); interstitial lung disease (ILD); interstitial pneumonia with autoimmune features (IPAF).

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Conflict of interest statement

Conflicts of Interest: None declared for Dr. Lee. Dr. Oldham has received speaking fees and sat on advisory boards for Genentech and Boehringer Ingelheim.

Figures

Figure 1
Figure 1
a) Axial and b) sagittal high resolution computed tomography (HRCT) views of organizing pneumonia (OP), part of the radiographic subdomain of interstitial pneumonia with autoimmune features (IPAF). Notable is airspace consolidation mainly in the periphery. c) Axial and d) sagittal HRCT views of nonspecific interstitial pneumonia (NSIP), which also qualifies under the IPAF radiographic subdomain. Ground glass opacities predominate, particularly in the basilar regions in this patient. Images courtesy of Dr. Jonathan Chung, associate professor of radiology, University of Chicago, Chicago, IL.
Figure 2
Figure 2
Kaplan–Meier survival curves of interstitial pneumonia with autoimmune features (IPAF), idiopathic pulmonary fibrosis (IPF) and connective tissue disease (CTD)-interstitial lung disease (ILD) cohorts. Overall a) IPAF cohort survival was significantly worse than the CTD-ILD cohort (p<0.001) and marginally better than the IPF cohort (p=0.07). After stratification of the IPAF cohort by the presence of a usual interstitial pneumonia pattern on high-resolution computed tomography and/or surgical lung biopsy b) IPAF patients without usual interstitial pneumonia (UIP) demonstrated survival similar to those with CTD-ILD (p=0.45), while those with UIP demonstrate survival similar to those with IPF (p=0.51). Reproduced with permission from the ©ERS 2015.
See this image and copyright information in PMC

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