A study from the Netherlands examined the outcomes for Primary Sclerosing Cholangitis (PSC) (Hepatology 2013; 2045-55). Using four independent hospital databases with 44 hospitals, allowed the investigators to identify 590 PSC patients from a population of almost 8 million. Median followup was 92 months. Mean age at diagnosis was 38.9 years.  A second comparison cohort of 450 patients was identified from three Dutch transplantation centers outside the study area.  134 (30%) of this cohort were present in the population-based cohort.  An IBD comparison group of 722 cases was identified as well from a population of 271,000 patients.

Results:

• Prevalence: 6 per 100,000.  This is significantly lower than previous estimates.  The authors emphasize the problem with previous epidemiology studies and the need for population-based studies with rigorous case-finding.
• Survival, estimated at 21.3 years for the entire cohort following diagnosis, was better than previous studies and this may indicate less selection bias than tertiary referral center studies.  It could also reflect diagnosis of milder cases with newer imaging techniques.
• 402 (68%) of PSC patients were diagnosed with inflammatory bowel disease.
• 23 (4%) had autoimmune hepatitis overlap.
• Colorectal cancer was 10-fold increased compared to ulcerative colitis controls and developed at a younger age (39 years compared with 59 years).
• Cholangiocarcinoma (CCA) was nearly 400-fold increased risk.  The cumulative risk of CCA was estimated at 20% after 30 years following PSC diagnosis.
• During followup, there were 97 deaths; 73 (75%) were PSC-related.

Related blog posts:

• PSC 2013 Review | gutsandgrowth
• Staying current with PSC | gutsandgrowth
• Colonic disease and PSC | gutsandgrowth
• Challenges with primary sclerosing cholangitis | gutsandgrowth Numerous references  with this blog post.
• Screening for subclinical PSC in IBD? | gutsandgrowth