Clinical–Liver, Pancreas, and Biliary TractThe Natural History of Small-Duct Primary Sclerosing Cholangitis
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)
- et al.
Natural history and prognostic variables in primary sclerosing cholangitis
Gastroenterology
(1991) - et al.
Primary sclerosing cholangitis: refinement and validation of survival models
Gastroenterology
(1992) - et al.
Clinical features in primary sclerosing cholangitis
Clin Liver Dis
(1998) - et al.
Hepatobiliary carcinoma associated with primary sclerosing cholangitis and chronic ulcerative colitis
Hum Pathol
(1985) - et al.
Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)
J Hepatol
(2002) - et al.
Small-duct primary sclerosing cholangitis:prevalence and natural history
Hepatology
(2002) - et al.
International autoimmune hepatitis group report: review of criteria for diagnosis of autoimmune hepatitis
J Hepatol
(1999) - et al.
Clinicopathologic features of the syndrome of primary sclerosing cholangitis
Gastroenterology
(1980) - et al.
Primary sclerosing cholangitis: a long-term follow-up study
Scand J Gastroenterol
(1987)
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Conflicts of interest: The authors have no conflicts of interest.