Incidence of primary sclerosing cholangitis: A systematic review and meta-analysis

Abstract

Incidence studies of primary sclerosing cholangitis (PSC) are important for describing the disease's burden and for shedding light on the disease's etiology. The purposes of this study were to conduct a systematic review of the incidence studies of PSC with a meta-analysis and to investigate possible geographic variations and temporal trends in the incidence of the disease. A systematic literature search of MEDLINE (1950MEDLINE ( -2010 and Embase (1980Embase ( -2010 was conducted to identify studies investigating the incidence of PSC. The incidence of PSC was summarized with an incidence rate (IR) and 95% confidence intervals. The test of heterogeneity was performed with the Q statistic. Secondary variables extracted from the articles included the following: the method of case ascertainment, the country, the time period, the age, the male/female incidence rate ratio (IRR), and the incidence of PSC subtypes (smallduct or large-duct PSC and inflammatory bowel disease). Stratified and sensitivity analyses were performed to explore heterogeneity between studies and to assess effects of study quality. Time trends were used to explore differences in the incidence across time. The search retrieved 1669 potentially eligible citations; 8 studies met the inclusion criteria. According to a random-effects model, the pooled IR was 0.77 (0.45-1.09) per 100,000 person-years. However, significant heterogeneity was observed between studies (P < 0.001). Sensitivity analyses excluding non-population-based studies increased the overall IR to 1.00 (0.82-1.17) and eliminated the heterogeneity between studies (P 5 0.08). The IRR for males versus females was 1.70 (1.34-2.07), and the median age was 41 years (35-47 years). All studies investigating time trends reported an overall increase in the incidence of PSC. Conclusion: The incidence of PSC is similar in North American and European countries and continues to increase over time. Incidence data from developing countries are lacking, and this limits our understanding of the global incidence of PSC. (HEPATOLOGY 2011;53:1590-1599

A Case of Rapidly Progressive Primary Sclerosing Cholangitis Requiring Liver Transplantation

“…According to a recent meta-analysis, its incidence was 0.77 per 100,000 person-years (1). It is a rare disease and seems to occur at lower frequencies in Asia and Southern Europe than in America and Northern Europe (2).…”

Primary Sclerosing Cholangitis in an Elderly Patient: A Diagnostic Challenge

“…PSC predominantly occurs in people aged between 37 and 43 years 2) . Its epidemiological charcterisics lead to a higher disease prevalence in the fourth and fifth decades of life.…”

“…Its epidemiological charcterisics lead to a higher disease prevalence in the fourth and fifth decades of life. A meta-analysis of data included from eight studies in Europe and North America reported that only one patient aged more than 84 years was diagnosed as having this disease 2,3) ; however, Japan is the only country that reported a second peak of incidence in the sixth decade of life. In its latest revision, the study found that 3 of 388 patients with PSC were older than 80 years 4) .…”

“…Japan is the only country that reported a second incidence peak in individuals aged 50-60 years 2) . It was first described in 1924 by Delbet, and it is associated with multiple complications, such as biliary tract stenosis, cholelithiasis, cholangitis, cholangiocarcinoma, and colon cancer in patients with concomitant ulcerative colitis 1) .…”

Sclerosing Cholangitis with Ulcerative Colitis after Bariatric Surgery

“…Hepatobiliary manifestations are much more commonly associated with ulcerative colitis (UC) than Crohn's disease (CD) [4,5]. The pathogenesis of hepatobiliary manifestations associated with UC may be related to liver gut interplay such as in primary sclerosing cholangitis (PSC), PSC/autoimmune hepatitis (AIH) overlap and IgG4 associated cholangitis (IAC).…”