The Natural History and Prognosis of Primary Biliary Cirrhosis with Clinical Features of Autoimmune Hepatitis

Fan Yang, Qixia Wang, Zhaoyue Wang, Qi Miao, Xiao Xiao, Ruqi Tang, Xiaoyu Chen, Zhaolian Bian, Haiyan Zhang, Yue Yang, Li Sheng, Jingyuan Fang, Dekai Qiu, Edward L. Krawitt, M. Eric Gershwin, Xiong Ma

Research output: Contribution to journalArticle

28 Scopus citations

Abstract

Although a variant of primary biliary cirrhosis (PBC) characterized by features of autoimmune hepatitis (AIH) has been recognized for many years, few studies with ample numbers of patients have focused on its natural history. This study aimed to clarify the natural history, prognosis, and response to therapy in a cohort of patients with PBC with AIH features. We retrospectively analyzed 277 PBC patients without AIH features and 46 PBC patients with AIH features seen between September 2004 and April 2014. The 5-year adverse outcome-free survival of PBC patients with AIH features was 58 % compared to 81 % in PBC patients without AIH features. Multivariate analysis in the patients with AIH features indicated that total bilirubin ≥2.70× the upper limit of normal predicted a poor prognosis (p = 0.008, relative risk 8.39, 95% confidence interval (CI) 1.73, 40.73). Combination therapy with ursodeoxycholic acid (UDCA) and immunosuppression provided better short-term responses in PBC patients with AIH features, defined by multiple criteria. Higher aspartate aminotransferase (AST) level at accession suggested better prognosis for PBC patients with AIH features while worse prognosis for PBC patients without AIH features. PBC patients with AIH features differ from those without AIH features in terms of natural history, prognostic indicators, and response to therapy.

Original languageEnglish (US)
Pages (from-to)114-123
Number of pages10
JournalClinical Reviews in Allergy and Immunology
Volume50
Issue number1
DOIs
StatePublished - Feb 1 2016

Keywords

  • Autoantibodies
  • Autoimmune hepatitis
  • Autoimmunity
  • Cholestasis
  • Overlap syndrome
  • Primary biliary cirrhosis

ASJC Scopus subject areas

  • Immunology and Allergy

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    Yang, F., Wang, Q., Wang, Z., Miao, Q., Xiao, X., Tang, R., Chen, X., Bian, Z., Zhang, H., Yang, Y., Sheng, L., Fang, J., Qiu, D., Krawitt, E. L., Gershwin, M. E., & Ma, X. (2016). The Natural History and Prognosis of Primary Biliary Cirrhosis with Clinical Features of Autoimmune Hepatitis. Clinical Reviews in Allergy and Immunology, 50(1), 114-123. https://doi.org/10.1007/s12016-015-8516-5