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Der Internist pp 765-777 | Cite as

Was ist gesichert in der Therapie der primär-biliären Zirrhose?

  • K. H. Wiedmann
  • P. Weber
  • W. Lauchart
Chapter

Zusammenfassung

Die primär-biliäre Zirrhose gehört zu den Leberkrankheiten, die im Zuge zunehmend subtilerer Diagnostik immer häufiger nachgewiesen werden; gleichzeitig hat diese Krankheit bei allerdings sehr variablem Verlauf letztendlich eine ungünstige Prognose. Kann diese mit modernen therapeutisch wirksamen Substanzen gebessert werden, oder profitieren die Patienten von einer Lebertransplantation? Der Aufsatz von Wiedmann und Mitarbeitern zieht eine aktuelle Bilanz.

Schlüsselwörter

Primär-biliäre Zirrhose Lebertransplantation Cholchizin Steroide Cyclosporin Chlorambucil Gallensäuren Azathioprin 

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Literatur

  1. 1.
    Bassendine MF, Macklon AF, Mulcaly R, James OFW (1982) Controlled trial of high and low dose D-penicillamine in primary biliary Cirrhosis. Gut 23:909Google Scholar
  2. 2.
    Berg PA, Klein R (1987) Immunology of primary biliary cirrhosis. Ballière’s Clin Gastroenterol 1:675CrossRefGoogle Scholar
  3. 3.
    Besedovsky HO, del Rey AE, Sorkin E (1985) Immune — neuroendocrine interactions. J Immunol 135:750Google Scholar
  4. 4.
    Bismuth H, Castaing D, Ericzon BG, Otte JB, Rolles K, Ringe B, Sloot M (1978) Hepatic Transplantation in Europe. Lancet II:674–676Google Scholar
  5. 5.
    Bloomer JR, Ghent CN (1981) Management of intractable cholestasis of primary biliary cirrhosis. Semin Liver Dis 1:345–353PubMedCrossRefGoogle Scholar
  6. 6.
    Bodenheimer HC, Schaffner F, Slernlieb I, Klion FM, Salvatore V, Pezullo J (1985) A prospective clinical trial of D-penicillamine in the treatment of primary biliary cirrhosis. Hepatology 5:1139–1142PubMedCrossRefGoogle Scholar
  7. 7.
    Bodenheimer H, Schaffner F, Pezzullo J (1986) Colchicine therapy in primary biliary cirrhosis. Hepatology 6:1172Google Scholar
  8. 8.
    Christensen E, Neuberger J, Crowe J, Altman DG, Popper H, Portmann B, Doniach D, Ranek L, Tygstrup N, Williams R (1985) Benefical effect of azathioprine and prediction of prognosis in primary biliary cirrhosis. Final results of an international trial. Gastroenterology 89:1084–1091PubMedGoogle Scholar
  9. 9.
    Cohen LB, Ambinder EP, Wolke AM, Field SP, Schaffner F (1985) Role of plasmapheresis in primary biliary cirrhosis. Gut 26:291–294PubMedCrossRefGoogle Scholar
  10. 10.
    Crowe J, Christensen E, Schmit M, Cochrane M, Ranek L, Watkinson G, Doniach D, Poper H, Tygstrup N, Williams R (1980) Azathioprine in primary biliary Cirrhosis. A preliminary report of an international trial. Gastroenterology 78:1005–1010PubMedGoogle Scholar
  11. 11.
    Cuthberth JA PAK CYC, Zerweck JE, Glass KD, Combes B (1984) Bone disease in primary biliary cirrhosis : increased bone resorption and turnover in the absence of osteoporis of osteomalacia. Hepatology 4:1–8CrossRefGoogle Scholar
  12. 12.
    David R, Kurtz W, Strohm WD, Leuschner U (1985) Die Wirkung von Ursodesoxycholsäure bei chronischen Lebererkrankungen: eine Pilotstudie. Z Gastroenterol 23:420Google Scholar
  13. 13.
    Davies M, Mawer EB, Klass HJ, Lumb GA, Berry JL, Warnes TW (1985) Vitamin D deficiency, osteomalacia, and primary biliary cirrhosis. Response to orally administered vitamin D 3. Dig Dis Sci 28:145–153CrossRefGoogle Scholar
  14. 14.
    Deering TB, Dickson R, Fleming R, Geal MG, McCall JT, Baggenstoss AH (1977) Effect of D-Penicilamine on copper retention in patients with primary biliary cirrhosis. Gastroenterology 72:1208–1212PubMedGoogle Scholar
  15. 15.
    Dickson ER, Fleming TR, Wiessner RH, Baldus WD, Fleming CR, Undwig UJ, McCall JT (1985) Trial of penicillamine in ad vanced primary biliary cirrhosis. N Engl J Med 312:1011–1015PubMedCrossRefGoogle Scholar
  16. 16.
    Doniach D, Roitt IM, Walker JG, Sherlock S (1966) Tissue antibodies in primary biliary cirrhosis, active chronic (luoid) hepatitis, Cryptogenic cirrhosis and other liver diseases and their clinical implications. Clin Exp Immunol 1:237–262PubMedCentralPubMedGoogle Scholar
  17. 17.
    Duncan JS, Kennedy HJ, Triger DR (1984) Treatment of pruritus due to chronic obstructive liver disease. Br Med J 289:22CrossRefGoogle Scholar
  18. 18.
    Epstein O, Villiers DD, Jain S, Potter BJ, Thomax HC, Sherlock S (1979) Reduction of immune complexes and immunoglobulins induced by D-penicillamine in primary biliary cirrhosis. N Engl J Med 300:274–278PubMedCrossRefGoogle Scholar
  19. 19.
    Epstein O, Jain S, Lee RG, Ross AM, Cook DG, Scheuer PJ, Sherlock S (1981) D-penicillamine treatment improves survival in primary biliary cirrhosis. Lancet I:1275–1277CrossRefGoogle Scholar
  20. 20.
    Epstein O, Chapman RWG, Lake Bakaar G (1982) The pancreas in primary biliary cirrhosis and primary sclerosing cholangitis. Gastroenterology 83:1177–1182PubMedGoogle Scholar
  21. 21.
    Epstein O, Kato Y, Dick R, Sherlock S (1982) Vitamin D, Hydroxyapatite and calcium gluconate in treatment of cortical bone thinning in post-menopausal women with primary biliary cirrhosis. Am J Clin Nutr 36:426–430PubMedGoogle Scholar
  22. 22.
    Epstein O, Cook DG, Jain S, McIntyre N, Sherlock S (1984) D-penicillamine and clinical trials in primary biliary cirrhosis. Heptology 4:1032Google Scholar
  23. 23.
    Esquivel CO, Van Thiel DH, Demetris AJ, Bernardos A, Iwatsuki S, Markus B, Gordon RD, Marsh JW, Makowka L, Tzakis AG (1988) Transplantation for primary biliary cirrhosis. Gastroenterology 94:1207–1216PubMedCentralPubMedGoogle Scholar
  24. 24.
    Fennell RH jr, Shikes RH, Vierling JM (1983) Relationship of pretransplant hepatobiliary disease to bile duct damage occurring in the liver allograft. Hepatology 3:84–89PubMedCrossRefGoogle Scholar
  25. 25.
    Finklestein M, Goldmann L, Grace ND, Folley M, Randall N (1987) Granulocytopenia complicating colchicine therapy for primary biliary cirrhosis. Gastroenterology 93:1231–1235PubMedGoogle Scholar
  26. 26.
    Fisher RL, Hofmann AF, Rossi S, Couverse JL, Lan SP (1986) Pathogenesis of morphological damage and major serum amino-transferase (AT) elevations in NCGS patients : Enhanced liver cell sensitivity — not defective lithocholate metabolism. Heptology 6:1169Google Scholar
  27. 27.
    Fisher MM, Paradine ME (1986) Influence of ursodesoxycholic acid (UDCA) on biochemical parameters in cholestatic liver disease. Gastroenterology 90:1725Google Scholar
  28. 28.
    Fonseca V, Epstein O, Gill DS, Kenon RK, Thomas M, McIntyre N, Dandona P (1987) Hyperparathyreoidism and low serum osteocalcin despite vitamin D replacement in primary biliary cirrhosis. J Clin Endocrinol Metab 64:873–877PubMedCrossRefGoogle Scholar
  29. 29.
    Ghent CM, Bloomer JR, Hsia YE (1978) Efficacy and safety of long term phenobarbital therapy of familial cholestasis. J Pediatr 93:127–132PubMedCrossRefGoogle Scholar
  30. 30.
    Ghent CN, Carruthers SG (1988) Treatment of Pruritus in primary biliary cirrhosis with rifampin. Gastroenterology 94:488–493PubMedGoogle Scholar
  31. 31.
    Haagsma EB, Manns M, Klein R, Grond J, Huizenga JR, Sloof MHJ, Meyer zum Büschenfelde KH, Perg PA, Grips CH (1987) Subtypes of antimitochondrial antibodies in primary biliary cirrhosis before and after orthotopic liver transplantation. Hepatology 7:129–133PubMedCrossRefGoogle Scholar
  32. 32.
    Hanid MA, Levi AJ (1980) Phototherapy for pruritus in primary biliary cirrhosis. Lancet II: 530CrossRefGoogle Scholar
  33. 33.
    Heathcote J, Ross A, Sherlock S (1976) A prospective controlled trial of azathio-prine in primary biliary cirrhosis. Gastroenterology 90:656–660Google Scholar
  34. 34.
    Herlong HF, Russel RM, Maddrey WS (1981) Vitamin A and zine therapy in primary biliary cirrhosis. Hepatology 1:348–351PubMedCrossRefGoogle Scholar
  35. 35.
    Herlong HF, Recker RR, Maddrey WC (1982) Bone disease in primary biliary cirrhosis: histologic features and response to 25-hydroxyvitamin D. Gastroenterology 82:103–108Google Scholar
  36. 36.
    Hodgson SF, Dickson ER, Wahner HW, Johnson KA, Mann KG, Rigss BL (1985) Bone loss and reduced osteoblast function in primary biliary cirrhosis. Ann Intern Med 103:855–860PubMedCrossRefGoogle Scholar
  37. 37.
    Hoffbauer FW (1960) Primary biliary cirrhosis: observations on the natural course of the disease in 25 women. Am J Dig Dis 5:348–383PubMedCrossRefGoogle Scholar
  38. 38.
    Hönsch HP, Balzer K, Dylewizc P, Kirsch W, Goebell H, Ohnhaus EE (1985) Effekt of rifampin treatment on hepatic drug metabolism and serum bile acids in patients with primary biliary cirrhosis. Eur J Clin Pharmacol 28:475–477CrossRefGoogle Scholar
  39. 39.
    Hoofnagle JH, Davis GL, Schafer DF, Peters M, Avisan MI, Pappas SC, Hausen RG, Minuk GY, Dusheiko GM, Campbell G, Mac Sween RNM, Jones EA (1986) Randomized trial of chlorambucil for primary biliary cirrhosis. Gastroenterology 91:1327–1334PubMedGoogle Scholar
  40. 40.
    Howat HT, Ralston AL, Varley H, Wilson JA (1966) The late results of long term treatment of primary biliary cirrhosis. Rev Int Hepatol 16:227–238PubMedGoogle Scholar
  41. 41.
    Javitt NG (1974) Timing of cholestyramin doses in cholestatic liver disease. N Engl J Med 290:1328–1329PubMedGoogle Scholar
  42. 42.
    Jeffrey GP, Müller DPR, Burrougles AK, Mathews S, Kemp C, Epstein O, Metclafe TA (1987) Vitamin E deficiency and its clinical significance in a dults with primary biliary cirrhosis and other forms of chronic liver disease. J Hepatol 4:307–317PubMedCrossRefGoogle Scholar
  43. 43.
    Kaplan MM, Alling DW, Zimmerman HJ, Wolfe HJ, Sepersky RA, Hirsch GS, Eita GH, Glick KA, Eagan KA (1986) A prospective trial of colchicine for primary biliary cirrhosis. N Engl J Med 315:1448–1454PubMedCrossRefGoogle Scholar
  44. 44.
    Karlsson-Parra A, Toettermann Th, Nyberg A, Mendel-Hartvig I, Loeoef L, Forsum U (1987) Immunological effects of Ciclosporin in primary biliary cirrhosis : suppression of activated T cells and autoantibody levels. Int Arch Allergy Appl Immunol 83:256–264PubMedCrossRefGoogle Scholar
  45. 45.
    Kershenobich D, Vargas F, Garcia-Tsao G, Tamayo RP, Gent M, Rojind M (1988) Cholchicine in the treatment of cirrhosis of the liver. N Engl J Med 318:1709–1713PubMedCrossRefGoogle Scholar
  46. 46.
    Knight RE, Bourne AJ, Newton M, Black A, Wilson P, Lawson MJ (1986) Neurologic syndrome associated with low levels of vitamin E in primary biliary cirrhosis. Gastroenterology 91:209–211PubMedGoogle Scholar
  47. 47.
    Krawitt EL, Grundman MJ, Mawer EB (1977) Absorption hydroxylation and excretion of vitamin D3 in primary biliary cirrhosis. Lancet II:1246–1249CrossRefGoogle Scholar
  48. 48.
    Lauchart W (1986) Ergebnisse der Lebertransplantation bei erwachsenen Zirrhosepatienten. Analyse verschiedener Klassifikationen und prognostischer Faktoren. Habilitationsschrift, HannoverGoogle Scholar
  49. 49.
    Leuschner U, Fischer H, Gatzen M, Kurtz W, Guldutusa S, Leuschner M, Hellstern A (1988) UDCA treatment of primary biliary cirrhosis: Results of a prospective double blind Trial. In: International Bile acid meeting. Trends in Bile acid research. Falk Symposium No 52Google Scholar
  50. 50.
    Lindgren S, Forsberg B, Eriksson S (1985) Observations during long-term plasma exchange in primary biliary cirrhosis. Scand J Gastroenterol 20:1124–1126PubMedCrossRefGoogle Scholar
  51. 51.
    Logan RFA, Ferguson A, Finlayson DC, Weir DG (1978) Primary biliary cirrhosis and coeliac disease: An association? Lancet I:230–233CrossRefGoogle Scholar
  52. 52.
    Matloffs DS, Alpert E, Resnick RH, Kaplan MM (1982) A prospective trial of D-penicillamine in primary biliary cirrhosis. N Engl J Med 306:319–326CrossRefGoogle Scholar
  53. 53.
    Matloff DS, Kaplan MM, Neer RM, Goldberg MJ, Bitman W, Wolfe HJ (1982) Osteoporosis in primary biliary cirrhosis : effects of 25-hydroxy vitamin D3 treatment. Gastroenterology 83:97–102PubMedGoogle Scholar
  54. 54.
    Mawer EB, Klass HJ, Warnes TW, Berry JL (1985) Metabolism of vitamin D in patients with primary biliary cirrhosis and alcoholic liver disease. Clin Sci 69:561–570PubMedGoogle Scholar
  55. 55.
    Hitchison HC, Bassendine MF, Record CO, Malcolm A, Watson A, James OFW (1986) Controlled trial of prednisolone for primary biliary cirrhosis: Good for the liver, bad for the bones. Hepatology 6:1211Google Scholar
  56. 56.
    Mitchison HC, Malcolm AJ, Bassendine MF, James OFW (1988) Metabolic bone disease in primary biliary cirrhosis at presentation. Gastroenterology 94:463–470PubMedGoogle Scholar
  57. 57.
    Miyazaki K, Nakayama F, Koga A (1984) Effect of chenodeoxycholic and ursodeoxycholic acids on isolated human hepatocytes. Dig Dis Sci 19:1123–1130CrossRefGoogle Scholar
  58. 58.
    Müller DPR, Lloyd JR, Wolff AL (1993) Vitamin E and neurological function. Lancet I:225–228Google Scholar
  59. 59.
    Neuberger J, Portman B, MacDougall BRD, Calne R, Williams R (1982) Recurrence of primary biliary cirrhosis after liver transplanttion. N Engl J Med 306:1–4PubMedCrossRefGoogle Scholar
  60. 60.
    Neuberger J, Christensen E, Portman B, Caballeria J, Rohes J, Ranck L, Tygstrup N, Williams R (1985) Double blind controlled trial of D-penicillamine in patients with primary biliary cirrhosis. Gut 26:114–119PubMedCrossRefGoogle Scholar
  61. 61.
    Neuberger J, Altmann DG, Christensen E, Tygstrupp N, Williams R (1986) Use of a prognostic index in evaluation of liver transplantation for primary biliary cirrhosis. Transplantation 41:713–716PubMedCrossRefGoogle Scholar
  62. 62.
    Perlstein SM (1981) Phototherapy for primary biliary cirrhosis (letter). Arch Dermatol 117:608PubMedCrossRefGoogle Scholar
  63. 63.
    Poupon P, Chrétien Y, Poupon RE, Ballet F, Camus Y, Darnis F (1987) Is urso-desoxycholic acid an effective treatment for primary biliary cirrhosis? Lancet I:834–836CrossRefGoogle Scholar
  64. 64.
    Poupon R, Poupon RE, Chrétien Y, Legendre C, Calmus Y, Ballet F (1988) Ursodeoxycholic acid (UDCA) for primary biliary cirrhosis (PBZ). In: X international Bile acid meeting Trends in Bile acid research. Falk Symposium No 52Google Scholar
  65. 65.
    Raedsch R, Stiehl A, Kommereil B (1988) Effects of ursodeoxycholic acid in primary biliary cirrhosis and primary sclerosing cholangitis. In: X International Bile acid meeting Trends in Bile acid research. Falk Symposium No 52Google Scholar
  66. 66.
    Reed JS, Meredith SC, Nemchausky BA, Rosenberg IH, Boyer JL (1980) Bone disease in primary biliary cirrhosis: reversal of osteomalacia with oral 25-hydroxy vitamin D. Gastroenterology 78:512–517PubMedGoogle Scholar
  67. 67.
    Roll J, Boyer JL, Barry D, Klatskin G (1983) The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 308:1–7PubMedCrossRefGoogle Scholar
  68. 68.
    Ros E, Garcia-Puges A, Reixach M, Cuso F, Rodes J (1984) Fat digestion and exocrine pancreatic function in primary biliary cirrhosis. Gastroenterology 87:180–187PubMedGoogle Scholar
  69. 69.
    Rosenblum JL, Keating JP, Prensky AL, Nelson JS (1981) A progressive neurological syndrome in children with chronic liver disease. N Engl J Med 304:503–508PubMedCrossRefGoogle Scholar
  70. 70.
    Routhier G, Epstein O, Janossy G, Thomas HC, Sherlock S (1980) Effects of cyclosporine A on suppression and inducer T Lymphocytes in primary biliary cirrhosis. Lancet I1:1223–1226CrossRefGoogle Scholar
  71. 71.
    Scheuer PJ (1980) Liver biopsy interpretation, 3 ed. Ballière Tindall, LondonGoogle Scholar
  72. 72.
    Shapiro JM, Smith H, Schaffner F (1979) Serum bilirubin: a prognostic factor in primary biliary cirrhosis. Gut 20:137–140PubMedCrossRefGoogle Scholar
  73. 73.
    Sherlock S (1959) Primary biliary cirrhosis (chronic intrahepatic obstructive janndice). Gastroenterology 37:574–586PubMedGoogle Scholar
  74. 74.
    Shepherd AN, Bedford GJ, Hil A, Bouchier IA (1984) Primary biliary cirrhosis, dark adaptometry, electro-oculography, and vitamin A state. Br Med J 289:1484–1485CrossRefGoogle Scholar
  75. 75.
    Taal BG, Schalm SW, Ten Kate FW, van Berge, Henegouwen GP, Brandt KH (1983) Low therapeutic value of D-penicillamine in short-term prospective trial in primary biliary cirrhosis. Liver 3:345–352PubMedCrossRefGoogle Scholar
  76. 76.
    Thomas HGL, Sherlock S (1952) Testosterone therapy for the pruritus of obstructive janndice. Br Med J 129:1289–1291CrossRefGoogle Scholar
  77. 77.
    Triger D, Manifold IN, Cloke P, Wunderwood JB (1980) D-penicillamine in primary biliary cirrhosis. Two year results. Gut 21:919–920Google Scholar
  78. 78.
    Volmer J (1982) The effectivity of immunosuppressive therapy on chronic aggressive (active) destructive cholangitis (PBZ) Morphometric investigations on the portal tracts of the liver. Liver 2:82–94PubMedCrossRefGoogle Scholar
  79. 79.
    Vogel W, Kathrein H, Judmaier G, Braunsteiner H (1988) Deterioration of primary biliary cirrhosis during treatment with ursodeoxycholic acid. Lancet I:1163CrossRefGoogle Scholar
  80. 80.
    Walt RP, Kemp CM, Lyness L, Bird AC, Sherlock S (1984) Vitamin A treatment for night blindness in primary biliary cirrhosis. Br Med J 288:1030–1031CrossRefGoogle Scholar
  81. 81.
    Walt RP, Daneshmend TK, Fellows IW, Toghill PJ (1988) Effect of stanazolol on itching in primary biliary cirrhosis. Br Med J 296:607CrossRefGoogle Scholar
  82. 82.
    Warnes TW, Smith A, Lee FI, Nagib Y, Haboubi, Johnson PJ, Hunt L (1987) A controlled trial of colchicine in primary biliary cirrhosis. J Hepatol 5:1–7PubMedCrossRefGoogle Scholar
  83. 83.
    Wiesner RH, Dickson ER, Lindor KD, Jorglusen R, La Russo NF, Baldus W (1987) A controlled clinical trial evaluating Cyclosporin in the treatment of primary biliary cirrhosis : a preliminary report. Hepatology 7:1025Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1988

Authors and Affiliations

  • K. H. Wiedmann
    • 1
  • P. Weber
    • 2
  • W. Lauchart
    • 3
  1. 1.Abteilung IMedizinische UniversitätsklinikTübingenDeutschland
  2. 2.Abteilung IMedizinischen UniversitätsklinikDeutschland
  3. 3.Abteilung für Allgemeine Chirurgie und PoliklinikChirurgischen Universitätsklinik TübingenDeutschland

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