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Multicenter Study
. 2002 Oct;51(4):562-6.
doi: 10.1136/gut.51.4.562.

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

C Y Ponsioen  1 , S M E Vrouenraets, W Prawirodirdjo, R Rajaram, E A J Rauws, C J J Mulder, J B Reitsma, S H Heisterkamp, G N J Tytgat
Affiliations
Multicenter Study

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

C Y Ponsioen et al. Gut. 2002 Oct.

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.

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Figures

Figure 1
Figure 1
Kaplan-Meier plot of cumulative survival of the entire primary sclerosing cholangitis cohort (n=174). Estimated median survival until death from liver disease or orthotopic liver transplantation was 18 years (95% confidence interval 15–21 years; broken lines).
Figure 2
Figure 2
Cholangiography showing type I intrahepatic and type 0 extrahepatic disease.
Figure 3
Figure 3
Cholangiography showing type II intrahepatic and type III extrahepatic disease.
Figure 4
Figure 4
Survival curves for intrahepatic disease (IHD) types 0–III. Type 0, n=9; type I, n=66; type II, n=40; type III, n=18. ERCP, endoscopic retrograde cholangiopancreatography.
Figure 5
Figure 5
Survival curves for extrahepatic disease (EHD) types 0–IV. Type 0, n=13; type I, n=22; type II, n=70; type III, n=17; type IV, n=10. ERCP, endoscopic retrograde cholangiopancreatography.
Figure 6
Figure 6
Kaplan-Meier cumulative survival curves and predicted survival curves according to the Cox model for the various categories of SUMIHDEHD‘‘ at mean of covariate AGEERCP (categories 1 and 5 for SUMIHDEHD‘‘ were not present in the cohort).
Figure 7
Figure 7
Estimated median survival as a function of the prognostic index before and after calibration.
See this image and copyright information in PMC

References

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