Primary Sclerosing Cholangitis Treatment Varies Significantly Among Care Centers

The survey of European hepatologists demonstrates the lack of clarity in guidelines.

A new survey of European health care centers with expertise in managing primary sclerosing cholangitis (PSC) suggests there is considerable intercenter variation in treatment and surveillance practices.

The authors of the report, published in JHEP Reports, say their data show there needs to be better harmonization of PSC treatment strategies.

PSC is an inflammatory disease of the biliary system that can lead to cirrhosis and end-stage liver disease, explained the authors of the new study. Seventy percent of patients with PSC also have inflammatory bowel disease (IBD), and patients with PSC are at a high risk of developing hepatobiliary or colorectal cancer.

Unfortunately, the study investigators said data are lacking on the best ways to care for patients with PSC, and international guidelines vary. Ursodeoxycholic acid (UDCA) is often used to treat PSC, but the investigators said its use is controversial. Immunosuppressive drugs and antibiotics are also sometimes prescribed, they said.

Given the lack of clarity of evidence and guidelines, they wanted to see whether there was any level of treatment uniformity among expert centers in Europe that treat patients with PSC.

In April 2020, they sent a PSC survey out to 278 hepatologists who were part of the PSC Study Group and the European Reference Network’s RARE-LIVER group. The recipients of the survey were from 17 European countries. They were asked 15 questions related to the size of their PSC cohorts, their treatment decisions, and their surveillance strategies for cholangiocarcinoma, gallbladder polyps, and IBD.

The results—based on responses from 82 physicians—showed wide variance from center to center. When it came to UDCA, half of physicians said they always prescribe the therapy to people with PSC, but 12% said they never do. Those who used the drug said they typically used it in patients with abnormally high alkaline phosphatase, severe changes in their PSC, pruritus, and IBD associated with PSC, or if the patient asked for it.

“Sixty-five percent of physicians continued UDCA treatment regardless of treatment response, while 23% of physicians discontinued UDCA if no biochemical or clinical response was observed,” the authors said.

A minority of physicians used other therapies, including vancomycin and immunosuppressives in select cases (12% of physicians and 37% of physicians, respectively).

The authors said the rates of medical treatments were generally similar, regardless of whether a particular treatment center treated a many or few patients with PSC.

Most physicians surveyed routinely screened patients with PSC for cholangiocarcinoma, mostly with MRI and ultrasound, the investigators found. However, physicians differed in how they treated patients in whom gallbladder polyps were detected.

“At detection of a gallbladder polyp of 6 mm, 46% of physicians recommended repeated ultrasound after 3 to 6 months, whereas 44% of physicians recommended immediate cholecystectomy,” the authors said.

In most cases (68%), physicians treating people with PSC without IBD referred patients for colonoscopies within 3 to 5 years, but 27% of physicians said they only referred patients for colonoscopies if they developed symptoms of IBD.

Taken together, the investigators said their survey responses show a lack of uniformity in PSC care in Europe.

“Overall, we found apparent uncertainties and discrepancies between practice and published guidelines and our results confirm that existing data and recommendations for clinicians are inadequate for uniform patient management, as shown by the overt heterogeneity in responses,” they said.

They added it will be challenging to align treatment practices more closely given the current variance in strategies, but they said the goal is important.

“Already established international networks and research associations should work to coordinate guideline recommendations in order to generate a better basis for developing clinical trials and to improve the daily management of patients with PSC,” they wrote.


Eliasson J, Lo B, Scramm C, et al. Survey uncovering variations in the management of primary sclerosing cholangitis across Europe. JHEP Rep. Published online August 14, 2022. doi:10.1016/j.jhepr.2022.100553

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