PMID- 34183525 OWN - NLM STAT- MEDLINE DCOM- 20211015 LR - 20211015 IS - 1531-6971 (Electronic) IS - 1070-5287 (Linking) VI - 27 IP - 5 DP - 2021 Sep 1 TI - Interstitial pneumonia with autoimmune features: from research classification to diagnosis. PG - 374-387 LID - 10.1097/MCP.0000000000000802 [doi] AB - PURPOSE OF REVIEW: The term interstitial pneumonia with autoimmune features (IPAF) was first proposed by an international task force in 2015 as a research classification to standardise nomenclature regarding patients with idiopathic interstitial pneumonia and features of connective tissue disease. However, how the use of this term and its proposed definition translates to clinical practice remains uncertain. This review will provide a comprehensive overview of studies of IPAF cohorts to date, discuss the consideration of IPAF as a distinct diagnostic entity and outline a suggested approach to patient management. RECENT FINDINGS: Considerable heterogeneity exists between published IPAF cohorts, with some cohorts exhibiting similarities to those with connective tissue disease-associated interstitial lung disease (CTD-ILD), and others more similar to idiopathic interstitial pneumonias including idiopathic pulmonary fibrosis (IPF). Little data exist to inform the management of patients who fulfil the IPAF criteria. Preliminary data supports pragmatic management of these patients as having a working clinical diagnosis of either idiopathic interstitial pneumonia or CTD-ILD. Future research studies into this approach are required. SUMMARY: The term IPAF, and its definition, have been of fundamental benefit to facilitating research in this diverse patient group. However, to date, there remain many unanswered questions regarding their natural histories and response to treatment. CI - Copyright (c) 2021 Wolters Kluwer Health, Inc. All rights reserved. FAU - Glenn, Laura M AU - Glenn LM AD - Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, NSW, Australia. AD - The University of Sydney School of Medicine (Central Clinical School), Sydney, NSW, Australia. FAU - Pugashetti, Janelle V AU - Pugashetti JV AD - Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of California, Davis, California, USA. FAU - Oldham, Justin AU - Oldham J AD - Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of California, Davis, California, USA. FAU - Corte, Tamera J AU - Corte TJ AD - Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown, NSW, Australia. AD - The University of Sydney School of Medicine (Central Clinical School), Sydney, NSW, Australia. AD - NHMRC Centre of Research Excellence in Pulmonary Fibrosis, Australia. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Curr Opin Pulm Med JT - Current opinion in pulmonary medicine JID - 9503765 SB - IM MH - *Autoimmune Diseases/diagnosis MH - *Connective Tissue Diseases/diagnosis MH - Humans MH - *Idiopathic Interstitial Pneumonias/diagnosis MH - *Idiopathic Pulmonary Fibrosis/diagnosis MH - *Lung Diseases, Interstitial/diagnosis EDAT- 2021/06/30 06:00 MHDA- 2021/10/16 06:00 CRDT- 2021/06/29 05:55 PHST- 2021/06/30 06:00 [pubmed] PHST- 2021/10/16 06:00 [medline] PHST- 2021/06/29 05:55 [entrez] AID - 00063198-202109000-00014 [pii] AID - 10.1097/MCP.0000000000000802 [doi] PST - ppublish SO - Curr Opin Pulm Med. 2021 Sep 1;27(5):374-387. doi: 10.1097/MCP.0000000000000802.