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A retrospective analysis including 769 PBC patients was conducted. The gender ratio was 6.1:1. Of the patients, 30.6% had one or more extrahepatic autoimmune (EHA) conditions. The proportion of patients with decompensated PBC at diagnosis increased from 25.0% in period 1 to 47.0% in period 4 (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.05). Of the 420 patients without complications on presentation, the Kaplan-Meier estimate revealed distinct outcomes between non-cirrhotic PBC and cirrhotic PBC, with estimated mean survival times of 145.1 months and 104.5 months, respectively (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). According to a subgroup analysis, gender and anti-mitochondrial antibody (AMA) status did not affect long-term prognosis, whereas patients with EHA conditions showed better prognoses. This study reveals evolving trends in male prevalence similar to their Western counterparts. Cirrhotic PBC patients were distinct from those with non-cirrhotic PBC at diagnosis based on difference in long-term outcome.<\/jats:p>","DOI":"10.1038\/s41598-017-06807-7","type":"journal-article","created":{"date-parts":[[2017,7,20]],"date-time":"2017-07-20T10:39:13Z","timestamp":1500547153000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Underestimated Male Prevalence of Primary Biliary Cholangitis in China: Results of a 16-yr cohort study involving 769 patients"],"prefix":"10.1038","volume":"7","author":[{"given":"Xiaoli","family":"Fan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tingting","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Shen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaotan","family":"Xi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3821-9342","authenticated-orcid":false,"given":"Li","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,7,26]]},"reference":[{"key":"6807_CR1","doi-asserted-by":"publisher","first-page":"1565","DOI":"10.1016\/S0140-6736(15)00154-3","volume":"386","author":"EJ Carey","year":"2015","unstructured":"Carey, E. 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