[Primary sclerosing cholangitis : Current diagnostics and treatment]. / Primär sklerosierende Cholangitis : Aktuelle Diagnostik und Therapie.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology. Characteristic features are multifocal strictures and dilatations of the bile ducts. In 60-80 % of cases the PSC is strongly associated with chronic inflammatory bowel disease, mostly in the form of pancolitis. The diagnosis is established based on detection of typical cholangiographic lesions of the bile ducts and exclusion of secondary causes of sclerosing cholangitis. There is no approved medical treatment, but in Germany ursodeoxycholic acid is frequently used. Clinically relevant stenoses can be successfully treated by interventional endoscopy. Patients with PSC suffer from a greatly increased risk of hepatobiliary malignancies, especially with respect to cholangiocarcinoma and colorectal cancer and therefore require regular surveillance and screening. Liver transplantation is currently the only curative treatment option.

Follow-up magnetic resonance imaging/3D-magnetic resonance cholangiopancreatography in patients with primary sclerosing cholangitis: challenging for experts to interpret.

BACKGROUND: In patients with primary sclerosing cholangitis follow-up magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP) is performed by many centres, particularly for the early detection of biliary malignancies and strictures. Clinically meaningful MRI-based definitions of primary sclerosing cholangitis related complications are, however, lacking. AIM: To investigate how primary sclerosing cholangitis experts interpret follow-up MRI/MRCP with a focus on conclusions that may impact clinical decision-making in primary sclerosing cholangitis. METHODS: Within the International Primary Sclerosing Cholangitis Study Group, an online survey on 16 real-life primary sclerosing cholangitis cases including clinical and biochemical information as well as a T2-weighted liver MRI/3D-MRCP was conducted. The interpretation of images and subsequent recommendations were assessed using a multiple-choice questionnaire. An inter-rater reliability calculation (Fleiss' kappa) was performed and factors potentially affecting the interpretation of magnetic resonance images were analysed using generalised linear mixed-effect models. RESULTS: Forty-four members/associates of the International Primary Sclerosing Cholangitis Study Group (median experience in the care of primary sclerosing cholangitis patients: 14 years) completed the survey. The MRI interpretation significantly varied among the participants. The lowest agreement was found with respect to the indication to perform subsequent endoscopic retrograde cholangiopancreatography (ERCP; Κ = 0.12, 95%CI 0.11-0.14). Elevated total bilirubin was the variable with the strongest effect on the rate of suspected dominant strictures, cholangiocarcinoma or ERCP recommendations. Liver cirrhosis did not prevent participants from recommending ERCP. Overall, the survey participants' recommendations contrasted the real-life management and outcome. CONCLUSIONS: In primary sclerosing cholangitis, the interpretation of follow-up MRI/3D-MRCP significantly varies even among experts and seems to be primarily affected by bilirubin levels. Generally accepted MRI-based definitions of primary sclerosing cholangitis-related complications are urgently needed.