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High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis

Olsson, Rolf; Glaumann, Hans; Almer, Sven; Broome, Ulrika; Lebrun, Barbro; Bergquist, Annika; Bjornsson, Einar; Prytz, Hanne LU ; Danielsson, Ake and Lindgren, Stefan (2009) In European Journal of Internal Medicine 20(2). p.190-196
Abstract
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... (More)
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. (C) 2008 European Federation of Internal Medicine. Published by Elsevier B.V (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Immunosuppression, Autoimmune hepatitis, Small duct PSC, Overlap syndrome, PSC, Ursodeoxycholic acid
in
European Journal of Internal Medicine
volume
20
issue
2
pages
190 - 196
publisher
Elsevier
external identifiers
  • wos:000265594900016
  • scopus:62649121481
ISSN
1879-0828
DOI
10.1016/j.ejim.2008.06.004
language
English
LU publication?
yes
id
f41c8fbb-c506-40bf-a482-5eea563aacf2 (old id 1427961)
date added to LUP
2009-06-25 13:19:07
date last changed
2017-10-22 03:44:51
@article{f41c8fbb-c506-40bf-a482-5eea563aacf2,
  abstract     = {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. (C) 2008 European Federation of Internal Medicine. Published by Elsevier B.V},
  author       = {Olsson, Rolf and Glaumann, Hans and Almer, Sven and Broome, Ulrika and Lebrun, Barbro and Bergquist, Annika and Bjornsson, Einar and Prytz, Hanne and Danielsson, Ake and Lindgren, Stefan},
  issn         = {1879-0828},
  keyword      = {Immunosuppression,Autoimmune hepatitis,Small duct PSC,Overlap syndrome,PSC,Ursodeoxycholic acid},
  language     = {eng},
  number       = {2},
  pages        = {190--196},
  publisher    = {Elsevier},
  series       = {European Journal of Internal Medicine},
  title        = {High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis},
  url          = {http://dx.doi.org/10.1016/j.ejim.2008.06.004},
  volume       = {20},
  year         = {2009},
}