Summary
We report a case of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome treated with cyclosporine A. Features of primary biliary cirrhosis were pruritus, high titer of antimitochondrial antibodies, inflammatory infiltrates surrounding interlobular bile ducts, and periportal granuloma. Features suggestive of autoimmune hepatitis were high titer of antinuclear antibodies, very high total immunoglobulins, and piecemeal necrosis. Because corticosteroids and ursodeoxycholic acid were inefficient, cyclosporine A was started at a dose of 3 mg/kg/day. A dramatic improvement in clinical condition, liver tests, and histology was noted. Discontinuation of cyclosporine A was followed by a clinical and histological relapse. Cyclosporine A reintroduction was again associated with a significant improvement. This case report suggests that in corticoresistant cases cyclosporine A could be an effective therapy for primary biliary cirrhosis-autoimmune hepatitis overlap syndrome.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Kaplan MM: Primary biliary cirrhosis. N Engl J Med 316:521–528, 1987
Culp KS, Fleming CR, Duffy J, Baldus WP, Dickson ER: Autoimmune associations in primary biliary cirrhosis. Mayo Clin Proc 57:365–370, 1982
Klöppel G, Seifert G, Lindner H, Dammermann R, Sack HJ, Berg PA: Histopathological features in mixed types of chronic aggressive hepatitis and primary biliary cirrhosis. Virchows Arch A Pathol Anat Histol 373:143–160, 1977
Geubel AP, Baggenstoss SH, Summerskill WHJ: Responses to treatment can differentiate chronic active liver disease with cholangitic features from the primary biliary cirrhosis. Gastroenterology 71:444–449, 1976
Hyams JS, Ballow M, Leichtner AM: Cyclosporine treatment of autoimmune chronic active hepatitis. Gastroenterology 3:890–893, 1987
Wiesner RH, Ludwig J, Lindor KD, Jorgensen RA, Baldus WP, Homburger HA, Dickson ER: A controlled trial of cyclosporine in the treatment of primary biliary cirrhosis. N Engl J Med 322:1419–1424, 1990
Lombard M, Portmann B, Neuberger J, Williams R, Tygstrup N, Ranek L, Ring-Larsen H, Rodes J, Navasa M, Trepo C, Pape G, Schou G, Badsberg JH, Andersen PK: Cyclosporine A treatment in primary biliary cirrhosis: results of a long-term placebo controlled trial. Gastroenterology 104:519–526, 1993
Doniach D, Walker JD: A unified concept of autoimmune hepatitis. Lancet 1:813–815, 1969
Carrougher JG, Schaffer RT, Canales LI, Goofman ZD: A 33-year-old woman with an autoimmune syndrome. Semin Liver Dis 11:256–262, 1991
Calmus Y, Weill B, Ozier Y, Chereau C, Houssin D, Poupon R: Immunosuppressive properties of bile acids: Differential effects of chenodeoxycholic (CDCA) and ursodeoxycholic (UDCA) acids on mouse mixed lymphocyte culture. Gastroenterology 103:617–620, 1992
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Duclos-Vallée, JC., Hadengue, A., Ganne-Carrié, N. et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Digest Dis Sci 40, 1069–1073 (1995). https://doi.org/10.1007/BF02064201
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02064201