Prognosis of primary sclerosing cholangitis in Israel is independent of coexisting inflammatory bowel disease

Henit Yanai, Shay Matalon, Amir Rosenblatt, Halim Awadie, Tania Berdichevski, Yifat Snir, Uri Kopylov, Lior Katz, Assaf Stein, Liat Mlynarsky, Hagit Tulchinsky, Fred Meir Konikoff, Shomron Ben Horin, Marius Braun, Ziv Ben-Ari, Yehuda Chowers, Yaacov Baruch, Oren Shibolet, Iris Dotan

Research output: Contribution to journalArticlepeer-review

Abstract

Background and aims: Primary sclerosing cholangitis (PSC) is a rare chronic progressive cholestatic disorder. We assessed its characteristics and natural history in Israel and compared its outcome with respect to coexisting inflammatory bowel disease (IBD). Methods: Data on characteristics, course and outcome were retrospectively retrieved on patients diagnosed with PSC from five large Israeli medical centers between 1988 and 2012. Patients with isolated PSC were compared with those with coexisting IBD to identify predictors of outcome. Results: Of 141 patients (56% males) with confirmed PSC, 98 (69.5%) had coexisting IBD. The average age at presentation was 38.8 ± 15.4 years and duration of follow-up was 7.93 ± 6.26 years. The crude estimated point prevalence was 4 cases per 105 persons. Demographics and clinical characteristics were similar among all patients except for symptoms at diagnosis (which were more prevalent among PSC-IBD patients) and utilization of multiple diagnostic modalities (which was more prevalent among isolated-PSC patients). More than one-third of the entire cohort had cirrhosis. A total of 15 patients (10.6%) died and 19 patients (13.5%) developed malignancy during follow-up. Nine patients (6.3%) underwent liver transplantation. Mean survival for the entire cohort was 26.3 ± 1.4 years and mean transplant-free survival was 23.5 ± 1.6 years. Cox proportionalhazard regression for transplantation or death revealed two independent risk factors: cirrhosis and malignancy [hazard ratio 4.25 (p = 0.004) and 2.58 (p = 0.046), respectively]. Conclusions: Survival rate of PSC patients in Israel is longer than reported rates worldwide and is independent of coexisting IBD.

Original languageEnglish
Pages (from-to)177-184
Number of pages8
JournalJournal of Crohn's and Colitis
Volume9
Issue number2
DOIs
StatePublished - 1 Feb 2015
Externally publishedYes

Keywords

  • Cirrhosis
  • Inflammatory bowel diseases
  • Liver transplantation
  • Malignancy
  • Primary sclerosing cholangitis
  • Survival

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