{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:07:54Z","timestamp":1787497674390,"version":"build-2736575974"},"reference-count":70,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2023,4,19]],"date-time":"2023-04-19T00:00:00Z","timestamp":1681862400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and Objectives: The \u201cinterstitial pneumonia with autoimmune features\u201d (IPAF) criteria have been criticized because of the exclusion of usual interstitial pneumonia (UIP) patients with a single clinical or serological feature. To classify these patients, the term UIPAF was proposed. This study aims to describe clinical characteristics and predictive factors for progression of a cohort of interstitial lung disease (ILD) patients with at least one feature of autoimmunity, applying criteria for IPAF, specific connective tissue diseases (CTD), and a definition of UIPAF when possible. Methods: We retrospectively evaluated data on 133 consecutive patients with ILD at onset associated with at least one feature of autoimmunity, referred by pulmonologists to rheumatologists from March 2009 to March 2020. Patients received 33 (16.5\u201369.5) months of follow-up. Results: Among the 101 ILD patients included, 37 were diagnosed with IPAF, 53 with ILD-onset CTD, and 11 with UIPAF. IPAF patients had a lower prevalence of UIP pattern compared to CTD-ILD and UIPAF patients (10.8% vs. 32.1% vs. 100%, p &lt; 0.01). During the follow-up, 4 IPAF (10.8%) and 2 UIPAF (18.2%) patients evolved into CTD-ILD. IPAF patients presented features not included in IPAF criteria, such as sicca syndrome (8.1%), and were more frequently affected by systemic hypertension (p &lt; 0.01). Over one year, ILD progression (greater extent of fibrosis on HRCT and\/or decline in PFTs) was less frequent in the IPAF group compared to CTD-ILD and UIPAF (32.3% vs. 58.8% vs. 72.7, p = 0.02). A UIP pattern and an IPAF predicted a faster (OR: 3.80, p = 0.01) and a slower (OR: 0.28, p = 0.02) ILD progression, respectively. Conclusions: IPAF criteria help identify patients who might develop a CTD-ILD, even though a single clinical or serological feature is respected. Future revisions of IPAF criteria should include sicca syndrome and separate UIP-pattern into a different definition (UIPAF), given its association with a different prognosis, independently from ILD classification.<\/jats:p>","DOI":"10.3390\/medicina59040794","type":"journal-article","created":{"date-parts":[[2023,4,20]],"date-time":"2023-04-20T03:25:11Z","timestamp":1681961111000},"page":"794","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria"],"prefix":"10.3390","volume":"59","author":[{"given":"Francesco","family":"Bozzao","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Azienda ULSS 2 \u201cMarca Trevigiana\u201d, 31100 Treviso, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Tomietto","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Rheumatology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1061-9507","authenticated-orcid":false,"given":"Elisa","family":"Baratella","sequence":"additional","affiliation":[{"name":"Institute of Radiology, Department of Medical Surgical and Health Sciences, Cattinara Hospital, University of Trieste, 34128 Trieste, Italy"},{"name":"Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Metka","family":"Kodric","sequence":"additional","affiliation":[{"name":"Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossella","family":"Cifaldi","sequence":"additional","affiliation":[{"name":"Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rossana","family":"Della Porta","sequence":"additional","affiliation":[{"name":"Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Prearo","sequence":"additional","affiliation":[{"name":"Vascular Medicine Unit, University Hospital LMU Munich, 81377 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia Maria Grazia","family":"Pirronello","sequence":"additional","affiliation":[{"name":"Emergency Medicine Unit, Azienda Ospedaliera Cannizzaro, 95126 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Confalonieri","sequence":"additional","affiliation":[{"name":"Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8990-859X","authenticated-orcid":false,"given":"Barbara","family":"Ruaro","sequence":"additional","affiliation":[{"name":"Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy"},{"name":"Pneumology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"Fischetti","sequence":"additional","affiliation":[{"name":"Internal Medicine Department, Rheumatology Unit, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"},{"name":"Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Fabris","sequence":"additional","affiliation":[{"name":"Department of Medicine, Surgery and Health Sciences, University of Trieste, 34128 Trieste, Italy"},{"name":"Internal Medicine Department, Azienda Sanitaria Universitaria Giuliano Isontina (ASUGI), 34128 Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,4,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.iac.2012.09.002","article-title":"Connective Tissue Disease\u2013Associated Lung Disease","volume":"32","author":"Olson","year":"2012","journal-title":"Immunol. 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