[Primary biliary cirrhosis: therapeutic trial using plasma exchange and immunosuppression. Preliminary results].
Four women aged 35 to 57 years (m: 49.2) with primary biliary cirrhosis and intractable pruritus had an estimated median survival time according to Christensen between 6 and 50 months (m: 27). They were enrolled in a therapeutic trial associating plasma exchange (PE) and immunosuppressive drugs. During the first 2 months, they received prednisone 15 mg/day, cyclophosphamide 2 mg/kg BW/day and 28 PE (60 ml/kg BW). Pruritus disappeared rapidly. After 2 months, mean levels of bilirubin, alkaline phosphatases, IgM and anti-mitochondrial antibodies dropped by 27 p. 100, 47 p. 100, 50 p. 100 and 85 p. 100 respectively, whereas amino-transferase and gamma-glutamyl-transpeptidase activities were unaltered. Two patients then received supportive therapy only: one was lost to follow-up after one year, the other died 50 months later from liver failure. The third patient received PE only when intractable pruritus reappeared: anti-mitochondrial antibodies, IgM and alkaline phosphatases remained below initial values for 38 months, until successful liver transplantation was performed. The fourth patient was treated on a long term basis by PE twice a month, prednisone 10 mg/day for 3 years and cyclophosphamide 1 mg/kgBW/day for one year only. Her initial estimated survival time was 6 months, but 3 years later she developed portal hypertension; anti-mitochondrial antibody titer was between 0 and 1/80, alkaline phosphatase levels reduced by 80 p. 100 to 70 p. 100; bilirubin level up by 50 p. 100. The mean survival time for the 4 patients exceeded 34 months, results better than those obtained with other kinds of therapy.
Rifle
G
G
Département de Néphrologie-Réanimation, Entérologie, Centre Hospitalier Universitaire, Dijon.
Mousson
C
C
Hillon
P
P
Marceau
M
M
Chalopin
J M
JM
Tanter
Y
Y
D000328:Adult; D000469:Alkaline Phosphatase / Q000097:blood; D000906:Antibodies / Q000032:analysis; D001663:Bilirubin / Q000097:blood; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000188:drug therapy / Q000628:therapy*; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology; D010951:Plasma Exchange / Q000379:methods; D011241:Prednisone / Q000627:therapeutic use*; D011537:Pruritus / Q000628:therapy
D000906:Antibodies; D003520:Cyclophosphamide; D000469:Alkaline Phosphatase; D001663:Bilirubin; D011241:Prednisone
Cirrhose biliaire primitive: essai thérapeutique par échanges plasmatiques et immunosuppression. Résultats préliminaires.