Gastroenterology

Gastroenterology

Volume 134, Issue 4, April 2008, Pages 975-980
Gastroenterology

Clinical–Liver, Pancreas, and Biliary Tract
The Natural History of Small-Duct Primary Sclerosing Cholangitis

https://doi.org/10.1053/j.gastro.2008.01.042Get rights and content

Background & Aims: The long-term prognosis of patients with small-duct primary sclerosing cholangitis (PSC) remains incompletely characterized. We aimed at determining the natural history and long-term outcomes of a large number of patients with small-duct PSC. Methods: Data from 83 patients with well-characterized small-duct PSC from several medical institutions in Europe and the United States were combined. Each patient with small-duct PSC was randomly matched to 2 patients with large-duct PSC by age, gender, calendar year of diagnosis, and institution. Results: The median age at diagnosis in both groups was 38 years (61% males). Nineteen (22.9%) of the 83 patients with small-duct PSC progressed to large-duct PSC in a median of 7.4 (interquartile range [IQR], 5.1–14) years. One patient with small-duct PSC who progressed to large-duct PSC was diagnosed with cholangiocarcinoma but after progression to large-duct PSC; 20 patients with large-duct PSC developed cholangiocarcinoma. Patients with small-duct PSC had a significantly longer transplantation-free survival compared with large-duct PSC patients (13 years [IQR, 10–17] vs 10 years [IQR, 6–14], respectively; hazard ratio, 3.04; 95% confidence interval: 1.82–5.06; P < .0001). Two patients with small-duct PSC who underwent liver transplantation had recurrence of small-duct PSC in the graft 9 and 13 years, respectively, after transplantation. Conclusions: Small-duct PSC is a disease of progressive potential but associated with a better long-term prognosis as compared with large-duct PSC. Small-duct PSC may recur after liver transplantation. Cholangiocarcinoma does not seem to occur in patients with small-duct PSC, unless the disease has progressed to large-duct PSC.

Section snippets

Materials and Methods

The results presented in the current study originate from cohorts of small-duct PSC patients from Norway, Sweden, the United Kingdom, and the United States.10, 11, 12 The criteria for identification of these patients; the diagnostic criteria; and the clinical, biochemical, and histologic variables assessed have previously been reported in detail.10, 11, 12 Briefly, the diagnostic criteria for small-duct PSC included (1) chronic cholestatic liver disease of unknown etiology; (2) percutaneous

General Characteristics of Patients With Small- and Large-Duct PSC

Among the 83 patients with small-duct PSC, 51 were male (61.5%) and 32 female (38.5%). In the large-duct group, 97 (61.8%) were male and 60 female (38.3%). Median age at diagnosis was 38 (IQR, 28–50) years and 38 (IQR, 28–48) years in the small-duct and the large-duct PSC group, respectively. The median duration of follow-up was 13 (IQR, 10–16) years among the small-duct patients, whereas the large-duct patients had a follow-up of 10 (IQR, 5.8–14) years (P = .002). A total of 72% of the

Discussion

The current series of a large number of patients with small-duct PSC and appropriately matched controls with large-duct PSC demonstrates that (1) patients with small-duct PSC follow a significantly better long-term prognosis as compared with large-duct PSC; (2) approximately a fourth of patients with small-duct PSC may progress to large-duct PSC within an average follow-up of 8 years; (3) cholangiocarcinoma does not seem to occur in patients with small-duct PSC; (4) patients with small-duct PSC

References (18)

There are more references available in the full text version of this article.

Cited by (231)

  • Prognostic models and autoimmune liver diseases

    2023, Best Practice and Research: Clinical Gastroenterology
  • Bile Duct Diseases

    2023, MacSween's Pathology of the Liver, Eighth Edition
View all citing articles on Scopus

Conflicts of interest: The authors have no conflicts of interest.

View full text