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Characterisation of patients with primary biliary cirrhosis responding to long term ursodeoxycholic acid treatment
  1. M Leuschnera,
  2. C F Dietricha,
  3. T Youa,
  4. C Seidlb,
  5. J Raedlea,
  6. G Herrmannc,
  7. H Ackermannd,
  8. U Leuschnera
  1. aCentre of Internal Medicine, Johann Wolfgang Goethe-Universität, Frankfurt am Main, Germany, bInstitute of Transfusion Medicine and Immunohematology of the RCBDS, cSenckenbergisches Institute of Pathology, dDepartment of Biomathematics
  1. Professor U Leuschner, Centre of Internal Medicine, Medical Clinic II, Johann Wolfgang Goethe-Universität, Theodor Stern Kai 7, D-60590 Frankfurt am Main, Germany


BACKGROUND In some patients with primary biliary cirrhosis, ursodeoxycholic acid causes full biochemical normalisation of laboratory data; in others, indexes improve but do not become normal.

AIMS To characterise complete and incomplete responders.

METHODS Seventy patients with primary biliary cirrhosis were treated with ursodeoxycholic acid 10–15 mg/kg/day and followed up for 6–13 years.

RESULTS In 23 patients (33%) with mainly stage I or II disease, cholestasis indexes and aminotransferases normalised within 1–5 years, except for antimitochondrial antibodies. Histological findings improved. Indexes were not normalised in 47 patients (67%) although the improvement of their biochemical functions parallelled the trend in the first group. In these incomplete responders histological findings improved to a lesser extent. The only difference between the two groups before treatment was higher levels of alkaline phosphatase and γ glutamyl transpeptidase in the incomplete responders. At onset of treatment the discriminant value separating responders from incomplete responders was 660 U/l for alkaline phosphatase and 131 U/l for γ glutamyl transpeptidase. One year later it was 239 and 27 U/l (overall predictive value for responders 92%, for incomplete responders 81%). There were no differences between the two groups concerning immune status, antimitochondrial antibody subtypes, liver histology, or any other data. HLA-B39, DRB1*08, DQB1*04 dominated in both groups.

CONCLUSIONS In patients with mainly early stages of primary biliary cirrhosis, higher values of alkaline phosphatase and γ glutamyl transpeptidase are the only biochemical indexes which allow discrimination between patients who will completely or incompletely respond to ursodeoxycholic acid treatment.

  • primary biliary cirrhosis
  • prognostic indexes
  • full response to ursodeoxycholic acid
  • incomplete responders
  • anti-p53 autoantibodies
  • HLA typing

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  • Abbreviations used in this paper:
    α1 fetoprotein
    alanine aminotransferase
    antimitochondrial antibodies
    antinuclear antibodies
    alkaline phosphatase
    aspartate aminotransferase
    AT III
    antithrombin III
    CA 19-9
    carbohydrate antigen 19-9
    γ glutamyl transpeptidase
    glutamate dehydrogenase
    hepatitis B surface antigen
    hepatitis C virus
    primary biliary cirrhosis
    smooth muscle antibodies
    ursodeoxycholic acid

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