{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T09:05:29Z","timestamp":1789376729578,"version":"build-2803163510"},"reference-count":20,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T00:00:00Z","timestamp":1787097600000},"content-version":"vor","delay-in-days":18,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T00:00:00Z","timestamp":1785542400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP22K06935"],"award-info":[{"award-number":["JP22K06935"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Primary biliary cholangitis (PBC) can be accompanied by interstitial lung disease (ILD) manifesting as granulomatous interstitial lung disease, but the mechanism of granuloma formation remains unclear. We report a case of PBC\u2010ILD coexisting with anti\u2010synthetase syndrome (ASS) in a 49\u2010year\u2010old woman who presented with persistent cough, facial rash, and arthritis. Laboratory tests demonstrated cholestatic enzyme elevation, anti\u2010mitochondrial M2 positivity, and anti\u2010aminoacyl\u2010tRNA synthetase antibody positivity, with elevated KL\u20106 and SP\u2010D levels. High\u2010resolution CT revealed lower\u2010lobe\u2010predominant ground\u2010glass opacities. Bronchoalveolar lavage fluid showed lymphocytosis with high CD4\/CD8 ratio. Liver biopsy confirmed PBC. Surgical lung biopsies revealed epithelial\u2010interface injury and focal apoptosis of bronchiolar and alveolar epithelium accompanied by CD4\u2010dominant T\u2010cell infiltration and non\u2010caseating granulomas (NCGs). Segment 2 showed cellular NSIP pattern and segment 9 showed fibrotic NSIP pattern. NCGs were more prominent in the cellular NSIP areas and decreased in the fibrotic NSIP areas. These findings suggest a possible pathological continuum from active inflammation by epithelial\u2010interface injury to fibrotic remodeling. These observations may highlight the potential relevance of generalized autoimmune epithelitis in the pathogenesis of PBC\u2010ILD.<\/jats:p>","DOI":"10.1111\/pin.70166","type":"journal-article","created":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T03:37:34Z","timestamp":1787197054000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary Biliary Cholangitis\u2010Associated Granulomatous Interstitial Lung Disease: Pulmonary Epithelial\u2010Interface Injury Supporting the Concept of Generalized Autoimmune Epithelitis"],"prefix":"10.1111","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-5244-5230","authenticated-orcid":false,"given":"Jiayao","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Diagnostic Pathology Asahikawa Medical University Hospital Asahikawa 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