PMID- 34937706 OWN - NLM STAT- MEDLINE DCOM- 20211224 LR - 20221115 IS - 1600-0617 (Electronic) IS - 0905-9180 (Print) IS - 0905-9180 (Linking) VI - 30 IP - 162 DP - 2021 Dec 31 TI - Interstitial pneumonia with autoimmune features: challenges and controversies. LID - 10.1183/16000617.0177-2021 [doi] LID - 210177 AB - The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration. CI - Copyright (c)The authors 2021. FAU - Mackintosh, John A AU - Mackintosh JA AUID- ORCID: 0000-0002-5254-4144 AD - Dept of Thoracic Medicine, The Prince Charles Hospital, Brisbane, Australia john.mackintosh@health.qld.gov.au. FAU - Wells, Athol U AU - Wells AU AD - Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Diseases, NHLI, Imperial College, London, UK. FAU - Cottin, Vincent AU - Cottin V AUID- ORCID: 0000-0002-5591-0955 AD - National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France. AD - Claude Bernard University Lyon 1, University of Lyon, INRAE, IVPC, UMR754, member of ERN-LUNG, Lyon, France. FAU - Nicholson, Andrew G AU - Nicholson AG AD - Dept of Histopathology, Royal Brompton Hospital, London, UK. AD - National Heart and Lung Institute, Imperial College, London, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AD - Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Diseases, NHLI, Imperial College, London, UK. LA - eng PT - Journal Article PT - Review DEP - 20211222 PL - England TA - Eur Respir Rev JT - European respiratory review : an official journal of the European Respiratory Society JID - 9111391 SB - IM CIN - Eur Respir Rev. 2021 Dec 22;30(162):. PMID: 34937707 MH - *Autoimmune Diseases/diagnosis MH - Humans MH - *Lung Diseases, Interstitial/diagnostic imaging/therapy PMC - PMC9488577 COIS- Conflict of interest: J.A. Mackintosh has nothing to disclose. Conflict of interest: A.U. Wells reports personal fees from Roche, Boehringer Ingelheim and Bayer outside the submitted work. Conflict of interest: V. Cottin reports personal fees and non-financial support from Actelion; grants, personal fees and non-financial support from Boehringer Ingelheim; personal fees from Bayer/MSD; personal fees and non-financial support from Roche/Promedior; and personal fees from Sanofi, Celgene/BMS, Galapagos, Galecto, Shionogi, AstraZeneca, Fibrogen, RedX and PureTech, outside the submitted work. Conflict of interest: A.G. Nicholson reports consulting fees received from Galapagos, Boehringer Ingelheim, Roche and Medical Quantitative Image Analysis in relation to idiopathic pulmonary fibrosis, outside the submitted work; and payment for educational activities in relation to ILD outside the submitted work. Conflict of interest: E.A. Renzoni reports receiving grants or contracts from Boehringer Ingelheim outside the submitted work; lecture fees paid to the institution from Boehringer Ingelheim, Roche and Chiesi outside the submitted work; and support for attending ATS conference from Boehringer Ingelheim outside the submitted work. EDAT- 2021/12/24 06:00 MHDA- 2021/12/25 06:00 PMCR- 2021/12/22 CRDT- 2021/12/23 05:33 PHST- 2021/07/24 00:00 [received] PHST- 2021/09/01 00:00 [accepted] PHST- 2021/12/23 05:33 [entrez] PHST- 2021/12/24 06:00 [pubmed] PHST- 2021/12/25 06:00 [medline] PHST- 2021/12/22 00:00 [pmc-release] AID - 30/162/210177 [pii] AID - ERR-0177-2021 [pii] AID - 10.1183/16000617.0177-2021 [doi] PST - epublish SO - Eur Respir Rev. 2021 Dec 22;30(162):210177. doi: 10.1183/16000617.0177-2021. Print 2021 Dec 31.