{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T09:49:04Z","timestamp":1762508944460,"version":"3.41.2"},"reference-count":12,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,2,16]],"date-time":"2023-02-16T00:00:00Z","timestamp":1676505600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>A subgroup of IPF patients can meet IPAF criteria (features suggesting an underlying autoimmune process without fulfilling established criteria for a CTD). This study was aimed to evaluate whether IPAF\/IPF patients compared to IPF patients differ in clinical profile, prognosis and disease course.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a retrospective, single center, case\u2013control study. We evaluated 360 consecutive IPF patients (Forl\u00ec Hospital, between 1\/1\/2002 and 28\/12\/2016) and compared characteristics and outcome of IPAF\/IPF to IPF.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty-two (6%) patients met IPAF criteria. IPAF\/IPF patients compared to IPF were <jats:bold>more frequently females<\/jats:bold> (<jats:italic>N<\/jats:italic> =\u20099\/22, 40.9% vs. <jats:italic>N<\/jats:italic> =\u200968\/338, 20.1%, <jats:italic>p<\/jats:italic> =\u20090.02), suffered more frequently from gastroesophageal reflux (54.5% vs. 28.4%, <jats:italic>p<\/jats:italic> =\u20090.01), and showed a higher prevalence of <jats:bold>arthralgias<\/jats:bold> (86.4% vs. 4.8%, <jats:italic>p<\/jats:italic> &amp;lt;\u20090.0001), <jats:bold>myalgias<\/jats:bold> (14.3% vs. 0.3%, <jats:italic>p<\/jats:italic> =\u20090.001) and <jats:bold>fever<\/jats:bold> (18.2% vs. 1.9%, <jats:italic>p<\/jats:italic> =\u20090.002). The serologic domain was detected in all cases (the most frequent were ANA in 17 and RF in nine cases) and morphologic domain (histology features) was positive in 6 out of 10 lung biopsies (lymphoid aggregates). Only patients with IPAF\/IPF evolved to CTD at follow-up (10\/22, 45.5%; six rheumatoid arthritis, one Sj\u00f6gren\u2019s and three scleroderma). The presence of IPAF was a positive prognostic determinant (HR 0.22, 95% CI 0.08\u20130.61, <jats:italic>p<\/jats:italic> =\u20090.003), whereas the isolated presence of circulating autoantibody did not impact prognosis (HR 1.00, 95% CI 0.67\u20131.49, <jats:italic>p<\/jats:italic> =\u20090.99).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The presence of IPAF criteria in IPF has a major clinical impact correlating with the risk of evolution to full blown-CTD during follow-up and identifying a subgroup of patients with a better prognosis.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fmed.2023.1087485","type":"journal-article","created":{"date-parts":[[2023,2,16]],"date-time":"2023-02-16T21:53:11Z","timestamp":1676584391000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Clinical implications of interstitial pneumonia with autoimmune features diagnostic criteria in idiopathic pulmonary fibrosis: A case control study"],"prefix":"10.3389","volume":"10","author":[{"given":"Sara","family":"Tomassetti","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Ravaglia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvia","family":"Puglisi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Athol U.","family":"Wells","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jay H.","family":"Ryu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcello","family":"Bosi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"Dubini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Piciucchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Girelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Parronchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federico","family":"Lavorini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisabetta","family":"Rosi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valentina","family":"Luzzi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco Matucci","family":"Cerinic","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Venerino","family":"Poletti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,2,16]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1295","DOI":"10.1183\/13993003.00019-2016","article-title":"Interstitial pneumonia with autoimmune features: the new consensus-based definition for this cohort of patients should be broadened","volume":"47","author":"Fischer","year":"2016","journal-title":"Eur Respir J"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"976","DOI":"10.1183\/13993003.00150-2015","article-title":"An official European Respiratory Society\/American Thoracic Society research statement: interstitial pneumonia with autoimmune features","volume":"46","author":"Fischer","year":"2015","journal-title":"Eur Respir J"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1016\/j.rmed.2016.10.017","article-title":"Interstitial pneumonia with autoimmune features: clinical, radiologic, and histological characteristics and outcome in a series of 57 patients","volume":"123","author":"Ahmad","year":"2017","journal-title":"Respir Med"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1016\/j.rmed.2019.03.011","article-title":"Clinical, serological and radiological features of a prospective cohort of interstitial pneumonia with autoimmune features (IPAF) patients","volume":"150","author":"Sambataro","year":"2019","journal-title":"Respir Med"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"1767","DOI":"10.1183\/13993003.01565-2015","article-title":"Characterisation of patients with interstitial pneumonia with autoimmune features","volume":"47","author":"Oldham","year":"2016","journal-title":"Eur Respir J"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","article-title":"Diagnosis of idiopathic pulmonary fibrosis. An official ATS\/ERS\/JRS\/ALAT clinical practice guideline","volume":"198","author":"Raghu","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"167","DOI":"10.1016\/j.rmed.2018.02.024","article-title":"Distinctive characteristics and prognostic significance of interstitial pneumonia with autoimmune features in patients with chronic fibrosing interstitial pneumonia","volume":"137","author":"Yoshimura","year":"2018","journal-title":"Respir Med"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"959","DOI":"10.1111\/resp.13379","article-title":"Usual interstitial pneumonia: a distinct group within interstitial pneumonia with autoimmune features? \u2013 reply","volume":"23","author":"Kelly","year":"2018","journal-title":"Respirology"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"1968","DOI":"10.1056\/NEJMoa1113354","article-title":"Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis","volume":"366","author":"Raghu","year":"2012","journal-title":"N Engl J Med"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"2125","DOI":"10.1007\/s10067-018-4111-5","article-title":"Clinical features, risk factors, and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study","volume":"37","author":"Dai","year":"2018","journal-title":"Clin Rheumatol"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"e020862","DOI":"10.1136\/bmjopen-2017-020862","article-title":"Prognostic significance of autoantibodies for idiopathic pulmonary fibrosis: protocol for a systematic review","volume":"8","author":"Kamiya","year":"2018","journal-title":"BMJ Open"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"731","DOI":"10.3346\/jkms.2013.28.5.731","article-title":"Clinical significance of serum autoantibodies in idiopathic interstitial pneumonia","volume":"28","author":"Kang","year":"2013","journal-title":"J Korean Med Sci"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2023.1087485\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,16]],"date-time":"2023-02-16T21:53:14Z","timestamp":1676584394000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2023.1087485\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,16]]},"references-count":12,"alternative-id":["10.3389\/fmed.2023.1087485"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2023.1087485","relation":{},"ISSN":["2296-858X"],"issn-type":[{"type":"electronic","value":"2296-858X"}],"subject":[],"published":{"date-parts":[[2023,2,16]]},"article-number":"1087485"}}