Semin Liver Dis 2000; 20(4): 481-496
DOI: 10.1055/s-2000-13157
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Recurrent Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and Autoimmune Hepatitis after Transplantation

Thomas W. Faust
  • Liver Study Unit, Section of Gastroenterology, Department of Medicine, The University of Chicago Hospitals and Clinics, The University of Chicago, Chicago, Illinois
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Publikationsverlauf

Publikationsdatum:
31. Dezember 2000 (online)

ABSTRACT

Viral hepatitis and malignancy frequently recur after transplantation, but recurrence of primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis is controversial. Differences in study design, number of patients, immunosuppressive treatment, length of follow-up, and criteria for recurrence account for discrepant results. Most patients with suspected recurrent disease are asymptomatic after transplantation. In patients transplanted for PBC, antimitochondrial antibodies frequently persist and do not correlate with disease recurrence; liver biopsy remains the gold standard for diagnosis. Exclusion of other disorders that can mimic PBC is paramount prior to making a diagnosis of recurrent disease. The effects of immunosuppression may modify or delay disease expression within the graft. If PBC recurs, intermediate-term patient and graft survival is excellent, but long-term studies will be necessary to address the impact of disease recurrence on the allograft. Due to lack of a diagnostic gold standard, a diagnosis of recurrent PSC after transplantation is difficult to make. An accurate diagnosis of PSC recurrence requires well-defined cholangiographic and histologic criteria. Other disorders that can produce biliary strictures after transplantation should be excluded. As with PBC, the effects of immunosuppression may modify or delay disease expression within the graft; medium-term patient and graft survival is excellent. Recurrence of autoimmune hepatitis is based on clinical, biochemical, serologic, and histologic criteria. As in patients transplanted for PBC and PSC, other conditions that can mimic autoimmune hepatitis require exclusion prior to making a diagnosis of recurrence. Most adult recipients respond to an increase in immunosuppression, whereas pediatric recipients do not respond as well. A cautious approach to withdrawal of immunosuppression is warranted in all patients transplanted for autoimmune hepatitis and the consequences of recurrent disease within the graft will require prolonged follow-up. Future studies should focus on preventive and therapeutic strategies for recurrent autoimmune diseases after transplantation.

REFERENCES

  • 1 Lee Y-M, Kaplan M M. Primary biliary cirrhosis. In: Friedman LS, Keefe EB, eds. Handbook of Liver Disease 1st ed. Edinburgh: Churchill Livingstone 1998: 197-214
  • 2 Van de Water J, Turchany J, Leung P SL. Molecular mimicry in primary biliary cirrhosis. Evidence for biliary epithelial expression of a molecule cross-reactive with pyruvate dehydrogenase complex-E2.  J Clin Invest . 1993;  91 2653-2664
  • 3 Scheuer P J, Lefkowitch J H. Primary biliary cirrhosis. In: Scheuer PJ, Lefkowitch JH, eds. Liver Biopsy Interpretation fifth ed. London: WB Saunders 1994: 51-58
  • 4 Batts K P, Wang X. Recurrence of primary biliary cirrhosis, autoimmune cholangitis and primary sclerosing cholangitis after liver transplantation.  Clin Liver Dis . 1998;  2 421-435
  • 5 Neuberger J, Portmann B, Macdougall B RD. Recurrence of primary biliary cirrhosis after liver transplantation.  N Engl J Med . 1982;  306 1-4
  • 6 Polson R J, Portmann B, Neuberger J. Evidence for disease recurrence after liver transplantation for primary biliary cirrhosis. Clinical and histologic follow-up studies.  Gastroenterology . 1989;  97 715-725
  • 7 Dietze O, Margreiter R. Primary biliary cirrhosis (PBC) after liver transplantation.  Transpl Proc . 1990;  22 1501-1502
  • 8 Hubscher S G, Elias E, Buckels J AC. Primary biliary cirrhosis. Histological evidence of disease recurrence after liver transplantation.  J Hepatol . 1993;  18 173-184
  • 9 Balan V, Batts K P, Porayko M K. Histological evidence for recurrence of primary biliary cirrhosis after liver transplantation.  Hepatology . 1993;  18 1392-1398
  • 10 Sebagh M, Farges O, Dubel L. Histological features predictive of recurrence of primary biliary cirrhosis after liver transplantation.  Transplantation . 1998;  65 1328-1333
  • 11 Van De Water J, Gerson L B, Ferrell L D. Immunohistochemical evidence of disease recurrence after liver transplantation for primary biliary cirrhosis.  Hepatology . 1996;  24 1079-1084
  • 12 Kim W R, Poterucha J J, Jorgensen R A. Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis?.  <~>Outcomes of ursodeoxycholic acid therapy and liver transplantation. Hepatology . 1997;  26 22-26
  • 13 Wong P YN, Portmann B, O'Grady J G. Recurrence of primary biliary cirrhosis after liver transplantation following fk506-based immunosuppression.  J Hepatol . 1993;  17 284-287
  • 14 Dmitrewski J, Hubscher S G, Mayer A D, Neuberger J M. Recurrence of primary biliary cirrhosis in the liver allograft: the effect of immunosuppression.  Hepatology . 1996;  24 253-257
  • 15 Mazariegos G V, Reyes J, Marino I R. Weaning of immunosuppression in liver transplant recipients.  Transplantation . 1997;  63 243-249
  • 16 Slapak G I, Saxena R, Portmann B. Graft and systemic disease in long-term survivors of liver transplantation.  Hepatology . 1997;  25 195-202
  • 17 Haagsma E B, Manns M, Klein R. Subtypes of antimitochondrial antibodies in primary biliary cirrhosis before and after orthotopic liver transplantation.  Hepatology . 1987;  7 129-133
  • 18 Klein R, Huizenga J R, Gips C H, Berg P A. Antimitochondrial antibody profiles in patients with primary biliary cirrhosis before orthotopic liver transplantation and titres of antimitochondrial antibody-subtypes after transplantation.  J Hepatol . 1994;  20 181-189
  • 19 Demetris A J, Markus B H, Esquival C. Pathologic analysis of liver transplantation or primary biliary cirrhosis.  Hepatology . 1988;  8 939-947
  • 20 Esquivel C O, Van Thiel H D, Demetris A J. Transplantation for primary biliary osis.  Gastroenterology . 1988;  94 1207-1216
  • 21 Buist L J, Hubscher S G, Vickers C. Does liver transplantation cure primary biliary cirrhosis?.  Transpl Proc . 1989;  21 2402
  • 22 Samuel D, Gugenheim J, Mentha G. Liver transplantation for primary biliary cirrhosis.  Transpl Proc . 1990;  22 1497-1498
  • 23 Gouw A SH, Haagsma E B, Manns M. Is there recurrence of primary biliary cirrhosis after transplantation?.  <~>A clinicopathologic study in long-term survivors. J Hepatol . 1994;  20 500-507
  • 24 Luettig B, Boeker K HW, Schoessler W. The antinuclear antibodies Sp100 and gp210 persist after orthotopic liver transplantation in patients with primary biliary cirrhosis.  J Hepatol . 1998;  28 824-828
  • 25 Dubel L, Farges O, Bismuth H. Kinetics of anti-M2 antibodies after liver transplantation for primary biliary cirrhosis.  Hepatology . 1995;  23 674-680
  • 26 Dubel L, Farges O, Courvalin J-C. Persistence of gp210 and multiple nuclear dots antibodies does not correlate with recurrence of primary biliary cirrhosis 6 years after liver transplantation.  J Hepatol . 1998;  28 169-170
  • 27 Ferrell L D, Lee R, Brixko C. Hepatic granulomas following liver transplantation.  Transplantation . 1995;  60 926-933
  • 28 Wiesner R H. Primary sclerosing cholangitis. In: Friedman LS, Keeffe EB, eds. Handbook of Liver Disease 1st ed. Edinburgh: Churchill Livingstone 1998: 215-225
  • 29 Sebagh M, Farges O, Kalil A. Sclerosing cholangitis following human orthotopic liver transplantation.  Am J Surg Pathol . 1995;  19 81-90
  • 30 McDonald V, Matalon T AS, Patel S. Biliary strictures in hepatic transplantation.  J Vasc Interv Radiol . 1991;  2 533-538
  • 31 Harrison R F, Davies M H, Neuberger J M, Hubscher S G. Fibrous and obliterative cholangitis in liver allografts: evidence of recurrent primary sclerosing cholangitis?.  Hepatology . 1994;  20 356-361
  • 32 Sheng R, Zajko A B, Campbell W L, Abu-Elmagd K. Biliary strictures in hepatic transplants: prevalence and types in patients with primary sclerosing cholangitis vs. those with other liver diseases.  Am J Roentgenol . 1993;  161 297-300
  • 33 Campbell W L, Sheng R, Zajko A B. Intrahepatic biliary strictures after liver transplantation.  Radiology . 1994;  191 735-740
  • 34 Sheng R, Campbell W L, Zajko A B, Baron R L. Cholangiographic features of biliary strictures after liver transplantation for primary sclerosing cholangitis: evidence of recurrent disease.  Am J Roentgenol . 1996;  166 1109-1113
  • 35 Narumi S, Roberts J P, Emond J C. Liver transplantation for sclerosing cholangitis.  Hepatology . 1995;  22 451-457
  • 36 Feller R B, Waugh R C, Selby W S. Biliary strictures after liver transplantation: clinical picture, correlates and outcomes.  J Gastroenterol Hepatol . 1996;  11 21-25
  • 37 Goss J A, Shackleton C R, Farmer D G. Orthotopic liver transplantation for primary sclerosing cholangitis. A 12-year single center experience.  Ann Surg . 1997;  225 472-483
  • 38 Jeyarajah D R, Netto G J, Lee S P. Recurrent primary sclerosing cholangitis after orthotopic liver transplantation.  Transplantation . 1998;  66 1300-1306
  • 39 Kita Y, Lake J R, Ferrell L D. Possible recurrence of primary sclerosing cholangitis following living-related liver transplantation: report of a case.  Transpl Proc . 1998;  30 3321-3323
  • 40 Saldeen K, Friman S, Olausson M, Olsson R. Follow-up after liver transplantation for primary sclerosing cholangitis: effects on survival, quality of life, and colitis.  Scand J Gastroenterol . 1999;  34 535-540
  • 41 Graziadei I W, Wiesner R H, Batts K P. Recurrence of primary sclerosing cholangitis following liver transplantation.  Hepatology . 1999;  29 1050-1056
  • 42 Marsh J W, Iwatsuki S, Makowka L. Orthotopic liver transplantation for primary sclerosing cholangitis.  Ann Surg . 1988;  207 21-25
  • 43 Letourneau J G, Day D L, Hunter D W. Biliary complications after liver transplantation in patients with preexisting sclerosing cholangitis.  Radiology . 1988;  167 349-351
  • 44 Lerut J, Demetris A J, Stieber A C. Intrahepatic bile duct strictures after human orthotopic liver transplantation.  Transpl Int . 1988;  1 127-130
  • 45 Ward E M, Kiely M J, Maus T P. Hilar biliary strictures after liver transplantation: cholangiography and percutaneous treatment.  Radiology . 1990;  177 259-263
  • 46 Haagsma E B, Mulder A HL, Gouw A SH. Neutrophil cytoplasmic autoantibodies after liver transplantation in patients with primary sclerosing cholangitis.  J Hepatol . 1993;  19 8-14
  • 47 Davern T J, Lake J R. Recurrent disease after liver transplantation.  Semin Gastrointest Dis . 1998;  9 86-109
  • 48 Czaja A. Autoimmune hepatitis. In: Friedman LS, Keefe EB, eds. Handbook of Liver Disease 1st ed. Edinburgh: Churchill Livingstone 1998: 63-83
  • 49 Neuberger J, Portmann B, Calne R, Williams R. Recurrence of autoimmune chronic active hepatitis following orthotopic liver grafting.  Transplantation . 1984;  37 363-365
  • 50 Wright H L, Bou-Abboud C F, Hassanein T. Disease recurrence and rejection following transplantation for autoimmune chronic active liver disease.  Transplantation . 1992;  53 136-139
  • 51 Birnbaum A H, Benkov K J, Pittman N S. Recurrence of autoimmune hepatitis in children after liver transplantation.  J Pediatr Gastroenterol Nutr . 1997;  25 20-25
  • 52 Sempoux C, Horsmans Y, Lerut J. Acute lobular hepatitis as the first manifestation of recurrent autoimmune hepatitis after orthotopic liver transplantation.  Liver . 1997;  17 311-315
  • 53 Ahmed M, Mutimer D, Hathway M. Liver transplantation for autoimmune hepatitis: a 12 year experience.  Transpl Proc . 1997;  29 496
  • 54 Prados E, Cuervas-Mons V, De La Mata M. Outcome of autoimmune hepatitis after liver transplantation.  Transplantation . 1998;  66 1645-1650
  • 55 Kerkar N, Hadzic N, Davies E T. De-novo autoimmune hepatitis after liver transplantation.  Lancet . 1998;  351 409-413
  • 56 Gotz G, Neuhaus R, Bechstein W O. Recurrence of autoimmune hepatitis after liver transplantation.  Transpl Proc . 1999;  31 430-431
  • 57 Narumi S, Hakamada K, Sasaki M. Liver transplantation for autoimmune hepatitis: rejection and recurrence.  Transpl Proc . 1999;  31 1955-1956
  • 58 Milkiewicz P, Hubscher S G, Skiba G. Recurrence of autoimmune hepatitis after liver transplantation.  Transplantation . 1999;  68 253-256
  • 59 Ratziu V, Samuel D, Sebagh M. Long-term follow-up after liver transplantation for autoimmune hepatitis: evidence of recurrence of primary disease.  J Hepatol . 1999;  30 131-141
  • 60 Sanchez-Urdazpal L, Czaja A J, van Hoek B. Prognostic features and role of liver transplantation in severe corticosteroid-treated autoimmune chronic active hepatitis.  Hepatology . 1992;  15 215-221