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Moreover, combined <jats:styled-content style=\"fixed-case\">PBC<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content> has been described in many case reports. Although neither the etiology of <jats:styled-content style=\"fixed-case\">PBC<\/jats:styled-content> nor that of <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content> has been elucidated, some genetic and immunological factors are known to be shared. Both disorders are autoimmune fibrotic diseases characterized by increased levels of profibrotic cytokines transforming growth factor \u03b2 (<jats:styled-content style=\"fixed-case\">TGF<\/jats:styled-content>\u03b2) and interleukin\u20106, which have recently been suggested to influence <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>\u2010helper 17 cells and regulatory <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content> cells involved in acquired immunity. <jats:styled-content style=\"fixed-case\">lcSSc<\/jats:styled-content> is accompanied by <jats:styled-content style=\"fixed-case\">CREST<\/jats:styled-content> symptoms, although complete <jats:styled-content style=\"fixed-case\">CREST<\/jats:styled-content> cases are rare, with relatively high prevalence of <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>aynaud's phenomenon, sclerodactyly and telangiectasia, and lower prevalence of calcinosis and esophageal dysmotility. Because patients with anticentromere antibody positive <jats:styled-content style=\"fixed-case\">PBC<\/jats:styled-content>\u2013<jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content> are at a high risk of developing portal hypertension, particular attention should be paid to the management of gastroesophageal varices. In addition, the management of <jats:styled-content style=\"fixed-case\">SSc<\/jats:styled-content>\u2010related non\u2010hepatic disorders, such as pulmonary fibrosis, pulmonary hypertension, heart disorder, infection and malignancy, is also important for improved outcomes. Because <jats:styled-content style=\"fixed-case\">PBC<\/jats:styled-content> is often complicated by rheumatic disease, hepatologists should keep the possibility of systemic disorder in mind when examining <jats:styled-content style=\"fixed-case\">PBC<\/jats:styled-content> patients.<\/jats:p>","DOI":"10.1111\/hepr.12285","type":"journal-article","created":{"date-parts":[[2013,12,6]],"date-time":"2013-12-06T10:21:57Z","timestamp":1386325317000},"page":"377-383","source":"Crossref","is-referenced-by-count":11,"title":["Pathophysiology and recent findings of primary biliary cirrhosis complicated by systemic sclerosis"],"prefix":"10.1111","volume":"44","author":[{"given":"Hiromasa","family":"Ohira","sequence":"first","affiliation":[{"name":"Department of Gastroenterology and Rheumatology Fukushima Medical University School of Medicine  Fukushima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Watanabe","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Rheumatology Fukushima Medical University School of Medicine  Fukushima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,2,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra043898"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-pathol-020712-164014"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-011-8253-3"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.50.4462"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1089-3261(03)00101-6"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-3-1"},{"key":"e_1_2_8_8_1","first-page":"202","article-title":"Scleroderma (systemic sclerosis): classification, subsets and pathogenesis","volume":"15","author":"LeRoy EC","year":"1988","journal-title":"J Rheumatol"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-010-8198-y"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.4103\/0019-5154.113930"},{"key":"e_1_2_8_11_1","first-page":"361","article-title":"Multiple teleangiectasi, Raynaud's phenomenon, sclerodactyly, and subcutaneus calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia","volume":"114","author":"Winterbauer RH","year":"1964","journal-title":"Bull Johns Hopkins Hosp"},{"key":"e_1_2_8_12_1","first-page":"423","article-title":"Progressive systemic sclerosis\u2010CREST syndrome observation on natural history and late complication in 90 patients","volume":"18","author":"Rodnan GP","year":"1975","journal-title":"Arthritis Rheum"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780270202"},{"key":"e_1_2_8_14_1","first-page":"1","article-title":"Primary biliary cirrhosis associated with systemic sclerosis: diagnostic and clinical challenges. 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