Elsevier

Journal of Hepatology

Volume 56, Issue 5, May 2012, Pages 1181-1188
Journal of Hepatology

Review
Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: A systematic review

https://doi.org/10.1016/j.jhep.2011.10.025Get rights and content
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open access

Summary

Objective

Studies on the epidemiology of primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) show variable outcome. We aimed at systematically reviewing the incidence and prevalence rates, as well as geographical distribution and temporal trends of PSC and PBC.

Data sources

A systematic search of literature was performed in Medline and EMBASE (search last conducted January 10th, 2011).

Study selection

Population-based epidemiological studies reporting incidence and/or prevalence rates for PSC or PBC in a defined geographical area of at least 100,000 adult inhabitants were considered relevant.

Data extraction

Study area, study period, number of patients, number of inhabitants, incidence per 100,000 inhabitants per year, prevalence per 100,000 inhabitants, method of case-finding, method of case-ascertainment, male/female ratio and in case of PSC, occurrence of inflammatory bowel diseases (IBD) were extracted from retrieved articles.

Results

The literature search yielded 2286 abstracts of which 31 articles fulfilled all inclusion criteria. Studies varied in size from 10 to 770 patients in catchment areas from 100,312 to 19,230,000 inhabitants. The incidence and prevalence rates for PSC range from 0 to 1.3 per 100,000 inhabitants/year and 0–16.2 per 100,000 inhabitants, respectively. PBC incidence rates range from 0.33 to 5.8 per 100,000 inhabitants/year and prevalence rates range from 1.91 to 40.2 per 100,000 inhabitants; prevalence rates are increasing in time.

Conclusions

Incidence and prevalence rates of both PSC and PBC vary widely and seem to be increasing. True population-based studies are scarce and therefore large population-based studies combining meticulous case-finding and case-ascertainment strategies are necessary.

Abbreviations

PSC
primary sclerosing cholangitis
PBC
primary biliary cirrhosis
IBD
inflammatory bowel disease
UDCA
ursodeoxycolic acid
MOOSE
Meta-analysis Of Observational Studies in Epidemiology
ICD
International Classification of Diseases
ERCP
endoscopic retrograde cholangiopancreatography
MRCP
magnetic resonance cholangiopancreatography
AMA
antimitochondrial antibodies

Keywords

Primary sclerosing cholangitis
Primary biliary cirrhosis
Epidemiology
Incidence
Prevalence

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