Primary Biliary Cholangitis Is Associated with CREST Syndrome

  • Kazumichi AbeEmail author
  • Hiromasa Ohira


Primary biliary cholangitis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Patients with PBC occasionally suffer complications from other autoimmune diseases. When PBC is associated with calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias (CREST) symptoms, it has been proposed to be a distinct clinical entity. Moreover, PBC associated with CREST syndrome has been described in many case reports. However, complete CREST cases are rare, with high prevalence of Raynaud’s phenomenon, sclerodactyly, and telangiectasias and lower prevalence of calcinosis and esophageal dysmotility. Because patients with anti-centromere antibody-positive PBC are at high risk of developing portal hypertension, particular attention should be paid to the management to gastroesophageal varices. This review provides a current overview of clinical characteristics and recent findings of PBC associated with CREST syndrome.


Primary biliary cholangitis CREST syndrome Anti-centromere antibody Nail-fold capillaroscopy 


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© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.Department of GastroenterologyFukushima Medical University School of MedicineFukushimaJapan

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