{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T15:56:23Z","timestamp":1790697383662,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T00:00:00Z","timestamp":1789430400000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pathology International"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Anti\u2010centromere antibody (ACA) positivity in primary biliary cholangitis (PBC) has been associated with a portal hypertension\u2010dominant clinical trajectory, even without advanced fibrosis. This study investigated whether porto\u2010sinusoidal vascular disorder (PSVD)\u2010spectrum portal venopathy may underlie this phenotype. This retrospective case\u2010series study included three ACA\u2010positive PBC\u2010spectrum patients who underwent liver biopsy. The clinical characteristics, imaging findings, liver and spleen stiffness, hepatic venography, and hepatic venous pressure gradient (HVPG) were evaluated. Liver specimens were assessed for PSVD\u2010spectrum portal venopathy and PBC\u2010related bile duct injury. All patients presented with PSVD\u2010spectrum portal venopathy on liver biopsy; PBC\u2010defining bile duct lesions were present in two patients, and the presence of ductular reaction was suggestive of a biliary disease but not diagnostic for PBC in one patient. All patients had manifestations of portal hypertension with preserved liver synthetic function and noncirrhotic liver stiffness. The portal sandwich sign was present in all patients. When available, spleen stiffness was markedly elevated, indicating spleen\u2013liver stiffness dissociation. HVPG was heterogeneous, with normal values in two patients and marked elevation in one patient. In summary, PSVD\u2010spectrum portal venopathy may contribute to disproportionate portal hypertension in ACA\u2010positive PBC\u2010spectrum patients but constitutes a diagnostic gray zone because PBC is defined as exclusionary in PSVD definitions.<\/jats:p>","DOI":"10.1111\/pin.70179","type":"journal-article","created":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T07:04:35Z","timestamp":1789455875000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Porto\u2010Sinusoidal Vascular Disorder\u2010Spectrum Portal Venopathy as a Structural Substrate for Portal Hypertension in Anti\u2010Centromere Antibody\u2010Positive Primary Biliary Cholangitis"],"prefix":"10.1111","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1449-0464","authenticated-orcid":false,"given":"Yuya","family":"Ando","sequence":"first","affiliation":[{"name":"Department of Gastroenterology National Defense Medical College Hospital 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