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Review
. 2022 Jun;24(6):213-226.
doi: 10.1007/s11926-022-01072-8. Epub 2022 Jun 1.

Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know

Elena K Joerns  1 , Traci N Adams  2 , Jeffrey A Sparks  3 , Chad A Newton  2 , Bonnie Bermas  4 , David Karp  4 , Una E Makris  4   5
Affiliations
Review

Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know

Elena K Joerns et al. Curr Rheumatol Rep. 2022 Jun.

Abstract

Purpose of review: This narrative review will focus on the role of the rheumatologist in evaluating patients with interstitial lung disease (ILD) without a defined rheumatic disease and will outline the current classification criteria for interstitial pneumonia with autoimmune features (IPAF) and describe what is known regarding IPAF pathobiology, natural history, prognosis, and treatment. Lastly, knowledge gaps and opportunities for future research will be discussed.

Recent findings: IPAF is a recently defined classification of ILD patients who have features suggesting an autoimmune-mediated process, but do not fulfill current rheumatic disease criteria. The goal of the IPAF criteria is to provide a uniform case definition for the study of autoimmune ILD patients who do not currently fit within standard ILD diagnostic categories, ultimately improving diagnosis and therapy. Many of these patients are referred for rheumatologic evaluation to aid the diagnostic process. The care of the IPAF patient is complex and is multidisciplinary with pulmonology, rheumatology, pathology, radiology, physical therapy, primary care, pulmonary transplant providers all serving vital roles. The rheumatologist has several roles which include classification, disease monitoring, and management.

Keywords: Interstitial lung disease; Interstitial pneumonia with autoimmune features; Pulmonary rheumatology collaboration; Rheumatologist evaluation.

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Conflict of interest statement

Dr. Elena K. Joerns reports salary support from a grant from Pfizer, Inc and Ruth L. Kirschstein Institutional National Research Service Award (T32).

Dr. Traci N. Adams reports no conflict of interest, financial or otherwise.

Dr. Jeffrey A. Sparks is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers R01 AR077607, P30 AR070253, and P30 AR072577) and the R. Bruce and Joan M. Mickey Research Scholar Fund. Dr. Sparks has received research support from Bristol Myers Squibb and performed consultancy for AbbVie, Boehringer Ingelheim, Bristol Myers Squibb, Gilead, Inova Diagnostics, Janssen, Optum, and Pfizer unrelated to this work. The funders had no role in the decision to publish or preparation of this manuscript. The content is solely the responsibility of the authors and does not necessarily represent the official views of Harvard University, its affiliated academic health care centers, or the National Institutes of Health.

Dr. Chad A. Newton reports career development award from the National Heart, Lung, and Blood Institute (K23HL148498) and have received consulting fees from Boehringer-Ingelheim in the amount less than $10,000.

Dr. Bonnie Bermas reports no conflict of interest, financial or otherwise.

Dr. David Karp reports no conflict of interest, financial or otherwise.

Dr. Una E. Makris reports funding by VA HSR&D and NIA for research unrelated to this work/manuscript with no other conflicts of interest.

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