Prognosis of Primary Sclerosing Cholangitis in Israel is Independent of Coexisting Inflammatory Bowel Disease
| Autor: | Assaf Stein, Halim Awadie, Yehuda Chowers, Lior H. Katz, Marius Braun, Shay Matalon, Yaacov Baruch, Oren Shibolet, Shomron Ben Horin, Amir Rosenblatt, Henit Yanai, Iris Dotan, Tania Berdichevski, Uri Kopylov, Liat Mlynarsky, Ziv Ben-Ari, Fred M. Konikoff, Hagit Tulchinsky, Yifat Snir |
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| Rok vydání: | 2014 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent medicine.medical_treatment Cholangitis Sclerosing Liver transplantation digestive system Gastroenterology Inflammatory bowel disease Primary sclerosing cholangitis Young Adult Risk Factors Internal medicine Prevalence medicine Humans Israel Child Survival rate Aged Retrospective Studies Endoscopic retrograde cholangiopancreatography medicine.diagnostic_test business.industry Incidence Retrospective cohort study General Medicine Middle Aged Inflammatory Bowel Diseases Prognosis medicine.disease Ulcerative colitis digestive system diseases Survival Rate Transplantation Female business |
| Zdroj: | Journal of Crohn's and Colitis. 9:177-184 |
| ISSN: | 1876-4479 1873-9946 |
| Popis: | 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 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. * Abbreviations: : PSC : primary sclerosing cholangitis; IBD : inflammatory bowel disease; CCA : cholangiocarcinoma; LT : liver transplantation; UC : ulcerative colitis; ERCP : endoscopic retrograde cholangiopancreatography; UDCA, ursodeoxycholic acid; MRCP : magnetic resonance cholangiopancreatography; CD : Crohn’s disease. |
| Databáze: | OpenAIRE |
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