Development and Validation of a Scoring System to Predict Outcomes of Patients with Primary Biliary Cirrhosis Receiving Ursodeoxycholic Acid Therapy

Willem J. Lammers, Gideon M. Hirschfield, Christophe Corpechot, Frederik Nevens, Keith Lindor, Harry L A Janssen, Annarosa Floreani, Cyriel Y. Ponsioen, Marlyn J. Mayo, Pietro Invernizzi, Pier M. Battezzati, Albert Parés, Andrew K. Burroughs, Andrew L. Mason, Kris V. Kowdley, Teru Kumagi, Maren H. Harms, Palak J. Trivedi, Raoul Poupon, Angela CheungAna Lleo, Llorenç Caballeria, Bettina E. Hansen, Henk R. Van Buuren

Research output: Contribution to journalArticle

142 Scopus citations


Background & Aims Approaches to risk stratification for patients with primary biliary cirrhosis (PBC) are limited, single-center based, and often dichotomous. We aimed to develop and validate a better model for determining prognoses of patients with PBC. Methods We performed an international, multicenter meta-analysis of 4119 patients with PBC treated with ursodeoxycholic acid at liver centers in 8 European and North American countries. Patients were randomly assigned to derivation (n = 2488 [60%]) and validation cohorts (n = 1631 [40%]). A risk score (GLOBE score) to predict transplantation-free survival was developed and validated with univariate and multivariable Cox regression analyses using clinical and biochemical variables obtained after 1 year of ursodeoxycholic acid therapy. Risk score outcomes were compared with the survival of age-, sex-, and calendar time-matched members of the general population. The prognostic ability of the GLOBE score was evaluated alongside those of the Barcelona, Paris-1, Rotterdam, Toronto, and Paris-2 criteria. Results Age (hazard ratio = 1.05; 95% confidence interval [CI]: 1.04-1.06; P 0.30 had significantly shorter times of transplant-free survival than matched healthy individuals (P

Original languageEnglish (US)
Pages (from-to)1804-1812e4
Issue number7
Publication statusPublished - Dec 1 2015



  • Autoimmune Liver Disease
  • Cholestasis
  • Predictive Factor
  • Prognosis

ASJC Scopus subject areas

  • Gastroenterology

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