Changes over a 20-year period in the clinical presentation of primary sclerosing cholangitis in Sweden.

OBJECTIVE

The use of magnetic resonance cholangiopancreaticography (MRCP) as a non-invasive diagnostic tool for primary sclerosing cholangitis (PSC), together with increased clinical awareness of the disease, has led to earlier diagnosis. The aim of this study was to investigate the clinical presentation of PSC including its association with inflammatory bowel disease (IBD) and the development of cholangiocarcinoma at one centre over an observation period of 20 years.

MATERIAL AND METHODS

All patients with well-defined PSC, diagnosed after 16 years of age and treated at Huddinge University Hospital between 1984 and 2004, were included in the study (n=246). PSC and IBD characteristics were retrieved from the patients' medical records. The patients were subdivided according to the date of diagnosis: 185 PSC patients diagnosed before 30 October 1998 were compared with 61 patients diagnosed after that date.

RESULTS

Patients diagnosed after 1998 were significantly older at diagnosis (mean age 41 versus 37 years) and presented fewer symptoms (47% versus 63%) as well as a lower frequency of coexisting IBD (69% versus 82%). In the whole group, women had significantly more symptoms than men, particularly pruritus (p<0.05).

CONCLUSIONS

The clinical spectrum of PSC in Sweden has changed over the past 20 years; today, PSC patients are older at diagnosis and associated IBD is less frequent.