Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population

Abstract

Background: Median survival of patients with primary sclerosing cholangitis (PSC) has been estimated to be 12 years. Cholangiography is the gold standard for diagnosis but is rarely used in estimating prognosis. Aims: To assess the natural history of Dutch PSC patients and to evaluate the prognostic value of a cholangiographic classification system. Patients: A total of 174 patients with established PSC attending a university hospital and three teaching hospitals from 1970 to 1999. Methods: Charts were reviewed for validity and time of diagnosis, concurrent inflammatory bowel disease, interventions, liver transplantation, occurrence of cholangiocarcinoma, and death. Follow up data were obtained from the charts and from the attending clinician or family physician. Median follow up was 76 months (range 1-300). The earliest available cholangiography was scored using a radiological classification system for the severity of sclerosis, developed in our institution. Survival curves were computed by the Kaplan-Meier method. Cholangiographic staging was used to construct a prognostic model, applying Cox proportional hazards analysis. Results: The estimated median survival from time of diagnosis to death from liver disease or liver transplantation was 18 years. Cholangiocarcinoma was found in 18 (10%) patients. Fourteen patients (8%) underwent liver transplantation. Cholangiographic scoring was inversely correlated with survival. A combination of intrahepatic and extrahepatic scoring, together with age at endoscopic retrograde cholangiopancreatography, proved strongly predictive of survival. Conclusions: The observed survival was considerably better than reported in earlier series from Sweden, the UK, and the USA. Classification and staging of cholangiographic abnormalities has prognostic value.

Metronidazole and Ursodeoxycholic Acid for Primary Sclerosing Cholangitis: A Randomized Placebo–Controlled Trial

“…14 We included the control ERCP in the study protocol because classification and staging of cholangiographic abnormalities has been shown to have prognostic value. 44 In those patients in whom comparable examinations with adequate filling were available, a trend showing either improvement or no progression of the disease was seen in the UDCA/MTZ group compared with those on UDCA monotherapy. However, no differences were evident dur-ing 3 years of therapy between the groups regarding referrals for liver transplantation, progression to decompensated liver cirrhosis, development of malignancy, or death.…”

Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline

“…Typical changes seen in PSC consist of minor irregularities of duct contour and local narrowing with prestenotic dilatation (type I), threadlike narrowings alternating with normal caliber of bile ducts or slight dilatation (type II), multiple strictures with saccular dilatations (type III), and the most advanced changes consisting of advanced ductal narrowing with resultant lack of filling of the peripheral ducts (type IV). The classification has later been modified by Majojie et al (21) and Ponsioen et al (22,23). The classification of Ponsioen et al (23) has been validated and shown to correlate with patient prognosis 6 ( Table 2).…”

Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

“…Median survival from time of diagnosis to death or liver transplantation was reported as 10 years (Farrant et al, 1991). In a Dutch cohort, transplantation free survival was 18 years on average (Ponsioen et al,2002 ). PSC patients are prone to end stage liver disease complications such as esophageal varices, ascites, and hepatic encephalopathy.…”