Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC)

https://doi.org/10.1016/S0168-8278(02)00036-3Get rights and content

Abstract

Background/Aims: This study aims at describing the natural history and outcome of small duct primary sclerosing cholangitis (PSC).

Methods: Thirty-two patients with small duct PSC were studied. The average time taken for diagnosis was 69 (1–168) months. The median follow-up time was 63 (1–194) months.

Results: All patients including one who underwent liver transplantation because of end-stage liver disease and hepatocellular carcinoma were alive at follow-up. None developed cholangiocarcinoma. In 27 patients repeated cholangiographic examinations were done after a median time of 72 (12–192) months from first ERCP. Four developed features of large duct PSC.

Conclusions: Small duct PSC rarely progresses to large bile duct PSC and it seems to have a benign course in most patients and no development of cholangiocarcinoma was found.

Introduction

The diagnosis of primary sclerosing cholangitis (PSC) is based on characteristic biochemical, histological and – most important – radiological features with irregularities and beadings of the intra- and/or extrahepatic bile ducts [1]. Some patients have the similar biochemical and histological features but a normal cholangiogram. For these the entity of small duct sclerosing cholangitis was introduced by Wee and Ludwig in 1985 and it was suggested that this may be part of the spectrum of PSC [2]. Since then, a few studies have been presented on this entity [3], [4]. However, the definition of small duct sclerosing cholangitis varies and some authors include patients having cholangiographic involvement of the intrahepatic biliary tree [4]. Others have argued that only patients with ulcerative colitis (UC), presence of chronic cholestasis, a normal cholangiogram and a liver biopsy consistent with PSC should be diagnosed as having small duct sclerosing cholangitis [5], [6]. On the other hand, only 70–80% of patients with large duct PSC have an associated inflammatory bowel disease (IBD), and the liver histology is characteristic for PSC in only 50% of all cases with typical large duct PSC [7], [8]. Thus, if all these strict criteria should be fulfilled when diagnosing small duct sclerosing cholangitis some patients might be missed. On the other hand, with less strict criteria it can be difficult to discriminate between other cholestatic disorders and small duct sclerosing cholangitis.

Besides diagnostic difficulties it is also unknown if patients with small duct sclerosing cholangitis may eventually progress to large bile duct PSC. It is also unknown whether the prognosis is comparable with that of large duct PSC and if patients with small duct sclerosing cholangitis also have an increased risk of developing cholangiocarcinoma. The aim of our retrospective study was to describe the natural history and outcome for patients of Swedish descent with small duct sclerosing cholangitis.

Section snippets

Patients

The study was performed by members of the Swedish Internal Medicine Liver Club (SILK) at seven hospitals throughout the country. This group had previously collected data concerning clinical features of 305 PSC patients [7], described the epidemiology of those with UC [9] and the group had also conducted treatment studies in PSC [10]. A tradition has evolved at these centers to look for and diagnose PSC and also to register all patients who have been suspected of having PSC but have not

General characteristics

Thirty-nine patients were initially reported to have small duct sclerosing cholangitis. After reevaluation of ERCPs, of liver biopsies and clinical data, seven patients were excluded. In two of these patients the ERCP did not fully disclose the intrahepatic biliary tree, in four patients some cholangiographic abnormalities were found and in one other patient the liver histology was not consistent with PSC. In seven patients the original ERCP had not been saved and could therefore not be

Discussion

The present study describes, the natural course of 32 patients with small duct sclerosing cholangitis. Twenty-seven of these had an additional cholangiographic investigation and only four developed signs of large duct PSC. Since one of these patients developed cirrhosis and had duct changes restricted to the intrahepatic biliary tree, it is difficult to exclude that the cholangiographic changes were not due to the cirrhotic changes [15]. In the majority of patients MRCP was used for follow-up

Acknowledgements

The authors acknowledge the financial support by Meda and Pharmacia companies.

References (23)

  • J Ludwig

    Small-duct primary sclerosing cholangitis

    Semin Liver Dis

    (1991)
  • Cited by (115)

    • Bile Duct Diseases

      2023, MacSween's Pathology of the Liver, Eighth Edition
    • Risk factors for chronic inflammatory disorders of ileal pouch

      2018, Pouchitis and Ileal Pouch Disorders: A Multidisciplinary Approach for Diagnosis and Management
    View all citing articles on Scopus
    View full text