The Burden of Large & Small Duct PSC in Adults & Children: A Population-Based Analysis

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The Burden of Large and Small Duct Primary Sclerosing Cholangitis in

Adults and Children: A Population-Based Analysis

Authors: Kaplan, Gilaad

G.; Laupland, B.; Butzner,

Decker; Urbanski, Stefan J.; Lee, S.

Source: The American Journal

of Gastroenterology, May 2007, pp. 1042-1049(8)

Publisher: Blackwell

Publishing

Objectives:

The epidemiology of primary sclerosing

cholangitis (PSC) has been incompletely assessed by

population-based studies. We therefore conducted a

population-based study to determine: (a) incidence rates of large and small

duct PSC in adults and children, ( the risk of inflammatory bowel disease on

developing PSC, and © patterns of clinical presentation with the advent of

magnetic resonance cholangiopancreatography (MRCP).

METHODS:

All residents of the Calgary Health Region diagnosed

with PSC between 2000 and 2005 were identified by medical records, endoscopic, diagnostic imaging, and pathology databases. Demographic and clinical information were obtained. Incidence rates were determined and risks associated with

PSC were reported as rate ratios (RR) with 95% confidence intervals (CI).

Forty-nine PSC patients were identified 

RESULTS:

For an age- and gender-adjusted annual incidence rate

of 0.92 cases per 100,000 person-years. The incidence

of small duct PSC was 0.15/100,000.  In

children the incidence rate was 0.23/100,000 compared with 1.11/100,000 in

adults.  PSC risk was similar in Crohn's

disease (CD; RR 220.0, 95% CI 132.4-343.7) and ulcerative colitis (UC; RR

212.4, 95% CI 116.1-356.5). Autoimmune hepatitis

overlap was noted in 10% of cases. MRCP diagnosed

large duct PSC in one-third of cases. Delay in

diagnosis was common (median 8.4 months). A minority

had complications at diagnosis: cholangitis (6.1%), pancreatitis (4.1%), and cirrhosis (4.1%).

Pediatric cases and small duct PSC are less common

than adult large duct PSC. Surprisingly, the risk of

developing PSC in UC and CD was similar.  Autoimmune hepatitis overlap was noted in a

significant minority of cases.   (Am J Gastroenterol

2007;102:1042-1049)

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