{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T18:53:44Z","timestamp":1788980024753,"version":"build-2803163510"},"reference-count":36,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2025,1,3]],"date-time":"2025-01-03T00:00:00Z","timestamp":1735862400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background\/Objectives: Anti-neutrophil cytoplasmic antibodies (ANCAs) have been found in interstitial lung disease (ILD) in recent years, although its impact on ILD prognosis is less known. To date, ANCAs are not included in the interstitial pneumonia with autoimmune features (IPAF) definition criteria. Therefore, ANCA-ILD, in the absence of known ANCA-associated vasculitis (AAV), could be underdiagnosed. Our aim was to analyze the clinical profile and prognosis of ANCA-ILD patients. Methods: Patients diagnosed with ILD and positive ANCA were enrolled in a retrospective, monocentric cohort study. Lung function outcomes and mortality were assessed according to clinical, serological, radiological, and treatment characteristics. Survival was analyzed using Kaplan\u2013Meier curves and Cox regression models. Results: A total of 23 patients were included, mostly women, with a median time from ILD diagnosis of 36 (24\u201368) months and a predominant anti-MPO pattern (56.5%). Nearly half of the patients had AAV, mostly microscopic polyangiitis (MPA). The presence of AAV was significantly associated with anti-MPO antibodies and an NSIP radiographic pattern. Overall, the fibrotic pattern (either UIP or fibrotic NSIP) was the most common (73.9%), mainly UIP (51.2%). However, it appeared less frequently in the AAV-ILD group. During follow-up, lung function impairment or radiological progression was observed in 65.2% of patients. Cumulative mortality incidence was high (43.4%), largely due to ILD itself (80%). A UIP pattern was associated with a higher and earlier mortality (HR 34.4 [1.36\u2013132]), while the use of immunosuppressants showed a trend towards lower ILD-related death. Conclusions: In our cohort, ANCA-ILD patients mostly presented with fibrotic patterns, with AAV in almost half of the cases and a high and early mortality rate, which suggests the need to assess ANCA in all ILD patients.<\/jats:p>","DOI":"10.3390\/jcm14010229","type":"journal-article","created":{"date-parts":[[2025,1,3]],"date-time":"2025-01-03T05:02:02Z","timestamp":1735880522000},"page":"229","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Clinical Profiles, Survival, and Lung Function Outcomes in ANCA-Associated Interstitial Lung Disease: An Observational Study"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2013-1545","authenticated-orcid":false,"given":"Cristina","family":"Valero-Mart\u00ednez","sequence":"first","affiliation":[{"name":"Rheumatology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0856-6290","authenticated-orcid":false,"given":"Claudia","family":"Valenzuela","sequence":"additional","affiliation":[{"name":"ILD Unit, Pulmonology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6825-7767","authenticated-orcid":false,"given":"Juan Pablo","family":"Baldivieso Ach\u00e1","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Mart\u00ednez-Besteiro","sequence":"additional","affiliation":[{"name":"ILD Unit, Pulmonology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Quiroga-Colina","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arantzazu","family":"Alfranca","sequence":"additional","affiliation":[{"name":"Immunology Unit, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"},{"name":"Medicine Department, School of Medicine, Universidad Aut\u00f3noma of Madrid, 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6823-4194","authenticated-orcid":false,"given":"Esther F.","family":"Vicente-Rabaneda","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"},{"name":"Medicine Department, School of Medicine, Universidad Aut\u00f3noma of Madrid, 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana Hern\u00e1ndez","family":"Mu\u00f1iz","sequence":"additional","affiliation":[{"name":"Radiology Unit, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7748-853X","authenticated-orcid":false,"given":"Santos","family":"Casta\u00f1eda","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9096-3780","authenticated-orcid":false,"given":"Rosario","family":"Garc\u00eda-Vicu\u00f1a","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital Universitario de La Princesa, IIS-Princesa, 28006 Madrid, Spain"},{"name":"Medicine Department, School of Medicine, Universidad Aut\u00f3noma of Madrid, 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1016\/j.cll.2019.07.003","article-title":"Antineutrophil Cytoplasmic Antibodies Testing and Interpretation","volume":"39","author":"Liang","year":"2019","journal-title":"Clin. 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