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High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis
Sahlgrens University Hospital.
Karolinska University Hospital.
Linköping University, Department of Clinical and Experimental Medicine, Gastroenterology and Hepatology . Linköping University, Faculty of Health Sciences. Östergötlands Läns Landsting, Centre for Medicine, Department of Endocrinology and Gastroenterology UHL.
Karolinska University Hospital.
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2009 (English)In: EUROPEAN JOURNAL OF INTERNAL MEDICINE, ISSN 0953-6205, Vol. 20, no 2, 190-196 p.Article in journal (Refereed) Published
Abstract [en]

Background: Overlap syndrome is a term used for overlapping features of autoimmune hepatitis and primary sclerosing cholangitis or primary biliary cirrhosis and for autoimmune cholangitis. We describe a high prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis.

Methods: We sought to retrieve all patients with overlap syndrome between primary sclerosing cholangitis and autoimmune hepatitis in six university hospitals in Sweden. The revised autoimmune hepatitis scoring system proposed by the International Autoimmune Hepatitis Group was used to establish the diagnosis autoimmune hepatitis. Endoscopic retrograde cholangiography and/or magnetic resonance cholangiography were used to separate the primary sclerosing cholangitis cases diagnosed through liver biopsy into small and large primary sclerosing cholangitis. A histologocial diagnosis compatible with both autoimmune hepatitis and primary sclerosing cholangitis was required for inclusion.

Results: 26 patients fulfilled our criteria for histological overlap of autoimmune hepatitis and primary sclerosing cholangitis, 7 (27%) of which had small duct primary sclerosing cholangitis. The reliability of the diagnosis small duct primary sclerosing cholangitis was supported by a very close similarity between small and large duct primary sclerosing cholangitis patients in clinical and laboratory data, and by a poor response to immunosuppressive therapy in the small duct primary sclerosing cholangitis patients. Patients with large duct overlap syndrome had a good response to immunosuppressive therapy. In both groups, our limited experience from ursodeoxycholic acid was largely poor.

Conclusions: Small duct primary sclerosing cholangitis is prevalent in the overlap syndrome between autoimmune hepatitis and primary sclerosing cholangitis.

Place, publisher, year, edition, pages
2009. Vol. 20, no 2, 190-196 p.
Keyword [en]
Overlap syndrome, PSC, Small duct PSC, Autoimmune hepatitis, Immunosuppression, Ursodeoxycholic acid
National Category
Medical and Health Sciences
Identifiers
URN: urn:nbn:se:liu:diva-18286DOI: 10.1016/j.ejim.2008.06.004OAI: oai:DiVA.org:liu-18286DiVA: diva2:217862
Available from: 2009-05-17 Created: 2009-05-15 Last updated: 2009-08-20

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Almer, Sven

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Gastroenterology and Hepatology Faculty of Health SciencesDepartment of Endocrinology and Gastroenterology UHL
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