Digestive Diseases and Sciences

, Volume 40, Issue 5, pp 1069–1073

Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome

Corticoresistance and effective treatment by cyclosporine A
  • Jean-Charles Duclos-Vallée
  • Antoine Hadengue
  • Nathalie Ganne-Carrié
  • Edith Robin
  • Claude Degott
  • Serge Erlinger
Liver: Infectious, Inflammatory, And Metabolic Disorders

DOI: 10.1007/BF02064201

Cite this article as:
Duclos-Vallée, JC., Hadengue, A., Ganne-Carrié, N. et al. Digest Dis Sci (1995) 40: 1069. doi:10.1007/BF02064201

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.

Key words

primary biliary cirrhosis-autoimmune hepatitis overlap syndromecorticoresistancecyclosporine A

Copyright information

© Plenum Publishing Corporation 1995

Authors and Affiliations

  • Jean-Charles Duclos-Vallée
    • 1
    • 2
  • Antoine Hadengue
    • 1
    • 2
  • Nathalie Ganne-Carrié
    • 1
    • 2
  • Edith Robin
    • 1
    • 2
  • Claude Degott
    • 1
    • 2
  • Serge Erlinger
    • 1
    • 2
  1. 1.From the Service d'Hépatologie and Unité de Recherches de Physiopathologie Hépatique (INSERM U24)Hôpital BeaujonFrance
  2. 2.Service d'Anatomie et de Cytologie PathologiquesHôpital BeaujonClichy, France
  3. 3.Service de GastroentérologieHôpital Cantonal UniversitaireGeneve 14France