{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T17:34:55Z","timestamp":1778607295250,"version":"3.51.4"},"reference-count":26,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,3,20]],"date-time":"2025-03-20T00:00:00Z","timestamp":1742428800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Anti\u2010neutrophil cytoplasmic antibodies (ANCA), a hallmark of systemic vasculitis (SV), have been reported in patients with idiopathic interstitial pneumonia (IIP). However, the clinical significance of ANCA in IIP remains unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively studied 101 IP patients diagnosed by pneumologists as idiopathic interstitial pneumonia (IIP,64) and IP with autoimmune features (IPAF,37). ANCA, anti\u2010myeloperoxidase and anti\u2010proteinase\u20103 were tested by immunofluorescence and ELISA. Chest HRCT patterns, pulmonary function tests (PFTs) and the evolution to SV during a 12\u2010month follow\u2010up were assessed. Multivariable regression analysis was performed to assess the association of baseline covariates with SV. The proximity of patients with close characteristics was investigated by cluster analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010one patients (20.8%) were ANCA+, similarly distributed between IPAF and IIP. ANCA+ patients were more likely to have NSIP (<jats:italic>p<\/jats:italic>\u2009=\u2009.02) and bronchiectasis (<jats:italic>p<\/jats:italic>\u2009=\u2009.02) on HRCT, less impaired 6MWD (<jats:italic>p<\/jats:italic>\u2009=\u2009.02), higher CRP (<jats:italic>p<\/jats:italic>\u2009=\u2009.02) and more arthralgias (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001) than ANCA\u2212 patients. During follow\u2010up, 9 (43%) p\u2010ANCA+ patients, but no ANCA\u2212 patients, developed SV (<jats:italic>p<\/jats:italic>\u2009=\u2009.001). p\u2010ANCA+ IP had 26.3 OR (95% CI 3.20\u201336.8) to evolve to SV within 12\u2009months (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.0001). Cluster analysis identified one group of 25 patients with significantly higher baseline NSIP (88%), p\u2010ANCA+ (48%), arthralgias (32%), and SV (24%) at 12\u2009months. Nevertheless, 12 p\u2010ANCA+ IP patients never developed SV.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ANCA+ IP patients had a high risk of developing SV and need close monitoring and prompt immunotherapy. ANCA+ IP patients not evolving to SV had a diagnosis of IIP or IPAF. These patients need longer observational studies to investigate if they represent a distinct ILD entity.<\/jats:p><\/jats:sec>","DOI":"10.1111\/eci.70025","type":"journal-article","created":{"date-parts":[[2025,3,20]],"date-time":"2025-03-20T07:44:37Z","timestamp":1742456677000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Anti\u2010neutrophil cytoplasmic antibodies associated interstitial pneumonia: A possible new clinical entity"],"prefix":"10.1111","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-5686-0127","authenticated-orcid":false,"given":"Claudia","family":"Iannone","sequence":"first","affiliation":[{"name":"ASST G. Pini\u2010CTO. UOC Clinica Reumatologica  Milan Italy"},{"name":"Department of Clinical Sciences and Community Health University of Milan  Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-1921-7370","authenticated-orcid":false,"given":"Maria Rosa","family":"Pellico","sequence":"additional","affiliation":[{"name":"ASST G. Pini\u2010CTO. 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