title: Prognosis of primary sclerosing cholangitis in israel is independent of coexisting inflammatory bowel Disease.
abstract: OBJECTIVE
  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 proportional hazard 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.
authors.forename: Henit
authors.initials: H
authors.lastname: Yanai
authors.forename: Shay
authors.initials: S
authors.lastname: Matalon
authors.forename: Amir
authors.initials: A
authors.lastname: Rosenblatt
authors.forename: Halim
authors.initials: H
authors.lastname: Awadie
authors.forename: Tania
authors.initials: T
authors.lastname: Berdichevski
authors.forename: Yifat
authors.initials: Y
authors.lastname: Snir
authors.forename: Uri
authors.initials: U
authors.lastname: Kopylov
authors.forename: Lior
authors.initials: L
authors.lastname: Katz
authors.forename: Assaf
authors.initials: A
authors.lastname: Stein
authors.forename: Liat
authors.initials: L
authors.lastname: Mlynarsky
authors.forename: Hagit
authors.initials: H
authors.lastname: Tulchinsky
authors.forename: Fred Meir
authors.initials: FM
authors.lastname: Konikoff
authors.forename: Shomron Ben
authors.initials: SB
authors.lastname: Horin
authors.forename: Marius
authors.initials: M
authors.lastname: Braun
authors.forename: Ziv
authors.initials: Z
authors.lastname: Ben-Ari
authors.forename: Yehuda
authors.initials: Y
authors.lastname: Chowers
authors.forename: Yaacov
authors.initials: Y
authors.lastname: Baruch
authors.forename: Oren
authors.initials: O
authors.lastname: Shibolet
authors.forename: Iris
authors.initials: I
authors.lastname: Dotan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000453:epidemiology; D007557:Israel / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D055815:Young Adult