Abstract
Prednisone alone or in combination with azathioprine is the established treatment of autoimmune hepatitis1-5. Remission occurs in 65% of treated patients within 2 years6,7 and immediate survival is improved8. The 10-year life expectancy of treated patients is 93% and is similar to that of an age- and sex-matched normal population9. Patients with histological cirrhosis at presentation have the same 10-year survival expectation as those without this finding (89% and 90%, respectively) and they respond similarly to initial therapy9. In contrast, untreated patients with disease of comparable severity have a 3-year survival of 50% and a 10-year life expectancy of 10%10. Prospective studies describe a mortality as high as 40% within 6 months8.
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References
Czaja AJ. Diagnosis, prognosis, and treatment of classical autoimmune chronic active hepatitis. In: Krawitt EL, Wiesner RH, editors. Autoimmune Liver Disease. New York: Raven Press; 1991:143–166.
Czaja AJ. Autoimmune hepatitis. Current therapeutic concepts. Clin Immunother. 1994;1:413–429.
Czaja AJ. Diagnosis and therapy of autoimmune liver disease. Med Clin N Am. 1996;80:973–994.
Czaja AJ. Autoimmune liver disease. In: Krawitt EL, Wiesner RH, editors. Treatment of Autoimmune Hepatitis. Amsterdam: Elsevier; 1998:499–515.
Czaja AJ. Drug therapy in the management of type 1 autoimmune hepatitis. Drugs. 1999;57:49–68.
Czaja AJ, Ammon HV, Summerskill WHJ. Clinical features and prognosis of severe chronic active liver disease (CALD) after corticosteroid-induced remission. Gastroenterology. 1980;78:518–523.
Czaja AJ, Beaver SJ, Shiels MT. Sustained remission following corticosteroid therapy of severe HBsAg-negative chronic active hepatitis. Gastroenterology. 1987;92:215–219.
Soloway RD, Summerskill WHJ, Baggenstoss AH et al. Clinical biochemical, and histological remission of severe chronic active liver disease: a controlled study of treatments and early prognosis. Gastroenterology. 1972;63:820–833.
Roberts SK, Therneau TM, Czaja AJ. Prognosis of histological cirrhosis in type 1 autoimmune hepatitis. Gastroenterology. 1996;110:848–857.
Geall MG, Schoenfield LJ, Summerskill WHJ. Classification and treatment of chronic active liver disease. Gastroenterology. 1972;63:458–465.
Hegarty JE, Nouri-Aria KT, Portmann B, Eddleston ALWF, Williams R. Relapse following treatment withdrawal in patients with autoimmune chronic active hepatitis. Hepatology. 1983;3:685–689.
Czaja AJ, Ludwig J, Baggenstoss AH, Wolf A. Corticosteroid-treated chronic active hepatitis in remission. Uncertain prognosis of chronic persistent hepatitis. N Engl J Med. 1981;304:5–9.
Schalm SW, Ammon HV, Summerskill WHJ. Failure of customary treatment in chronic active liver disease: causes and management. Ann Clin Res. 1976;8:221–227.
Davis GL, Czaja AJ, Ludwig J. Development and prognosis of histologic cirrhosis in corticosteroid-treated HBsAG-negative chronic active hepatitis. Gastroenterology. 1984;87:1222–1227.
Czaja AJ, Davis GL, Ludwig J, Taswell HE Complete resolution of inflammatory activity following corticosteroid treatment of HBsAg-negative chronic active hepatitis. Hepatology. 1984;4:622–627.
Czaja AJ. Chronic active hepatitis: the challenge for a new nomenclature. Ann Intern Med. 1993;119:510–517.
Czaja AJ. The variant forms of autoimmune hepatitis. Ann Intern Med. 1996;125:588–598.
Czaja AJ. Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatology. 1998;28:360–365.
Czaja AJ. Variant forms of autoimmune hepatitis. Curr Gastroenterol Rep. 1999;1:63–70.
Czaja AJ, Rakela J, Ludwig J. Features reflective of early prognosis in corticosteroid-treated severe autoimmune active hepatitis. Gastroenterology. 1988;95:448–453.
Schalm SW, Korman MG, Summerskill WHJ, Czaja AJ, Baggenstoss AH. Severe chronic active liver disease. Prognostic significance of initial morphologic patterns. Am J Dig Dis. 1977;22:973–980.
Cooksley WGE, Bradbear RA, Robinson W et al. The prognosis of chronic active hepatitis without cirrhosis in relation to bridging necrosis. Hepatology. 1986;6:345–348.
Summerskill WHJ, Korman MG, Ammon HV, Baggenstoss AH. Prednisone for chronic active liver disease: dose titration, standard dose, and combination with azathioprine compared. Gut. 1975;16:876–883.
Liberman UA, Weiss SR, Broil J et al. Effect of oral alendronate on bone mineral density and the incidence of fractures in portmenopausal osteoporosis. N Engl J Med. 1995;333:1437–1443.
Guanabens N, Pares A, Monegal A et al. Etidronate versus fluoride for treatment of osteopenia in primary biliary cirrhosis: preliminary results after 2 years. Gastroenterology. 1997;113:219–224.
Wolfhagen FHJ, Van Buuren HR, denOuden JW et al. Cyclical etidronate in the prevention of bone loss in corticosteroid-treated primary biliary cirrhosis. A prospective, controlled pilot study. J Hepatol. 1997;26:325–330.
Lindor KD, Jorgenson RA, Petz JL, Dickson ER. Etidronate for cholestasis induced osteoporosis: a randomized trial. Gastroenterology. 1999;116:A1239 (abstract).
Czaja AJ, Wolf AM, Baggenstoss AH. Laboratory assessment of severe chronic active liver disease (CALD): correlation of serum transaminase and gamma globulin levels with histologic features. Gastroenterology. 1981;80:687–692.
Czaja AJ. Low dose corticosteroid therapy after multiple relapses of severe HBsAg-negative chronic active hepatitis. Hepatology. 1990;11:1044–1049.
Johnson PJ, McFarlane IG, Williams R. Azathioprine for long-term maintenance of remission in autoimmune hepatitis. N Engl J Med. 1995;333:958–963.
Ben Ari Z, Mehta A, Lennard L, Burroughs AK. Azathioprine-induced myelosuppression due to thiopurine methyltransferase deficiency in a patient with autoimmune hepatitis. J Hepatol. 1995;23:351–354.
Yates CR, Krynetski EY, Loennechen T et al. Molecular diagnosis of thiopurine S-methyltrans-ferase deficiency: genetic basis for azathioprine and mercaptopurine intolerance. Ann Intern Med. 1997;126:608–614.
Pratt DS, Flavin DP, Kaplan MM. The successful treatment of autoimmune hepatitis with 6-mer-captopurine after failure with azathioprine. Gastroenterology. 1996;110:271–274.
Sanchez-Urdazpal L, Czaja AJ, Van Hoek B, Krom RAF, Wiesner RH. Prognostic features and role of liver transplantation in severe corticosteroid-treated autoimmune chronic active hepatitis. Hepatology. 1992;15:215–221.
Ratziu V, Samuel D, Sebagh M et al. Long-term follow-up after liver transplantation for autoimmune hepatitis: evidence of recurrence of primary disease. J Hepatol. 1999;30:131–141.
Gonzalez-Koch AR, Czaja AJ, Carpenter HA et al. Orthotopic liver transplantation for autoimmune hepatitis: long-term outcome and incidence of disease recurrence. Hepatology. 1999;30:427A.
Sempoux C, Horsmans Y, Lerut J, Rahier J, Geubel A. Acute lobular hepatitis as the first manifestation of recurrent autoimmune hepatitis after orthotopic liver transplantation. Liver. 1997;17:311–315.
Neuberger J, Portmann B, Calne R, Williams R. Recurrence of autoimmune chronic active hepatitis following orthotopic liver grafting. Transplantation. 1984;37:363–365.
Devlin J, Donaldson P, Portmann B, Heaton N, Tan K-C, Williams R. Recurrence of autoimmune hepatitis following liver transplantation. Liver Transplant Surg. 1995;1:162–165.
Wright HL, Bou-Abboud CF, Hassanein T et al. Disease recurrence and rejection following liver transplantation for autoimmune chronic active liver disease. Transplantation. 1992;53:136–139.
Hayashi M, Keefe EB, Krams SM et al. Allograft rejection after liver transplantation for autoimmune liver disease. Liver Transplant Surg. 1998;4:208–214.
Thomson AW, Carroll PB, McCauley J et al. FK 506: a novel immunosuppressant for treatment of autoimmune disease. Rationale and preliminary clinical experience at the University of Pittsburgh. Springer Semin Immunopathol. 1993;14:323–344.
Van Thiel DH, Wright H, Carroll P et al. Tacrolimus: a potential new treatment for autoimmune chronic active hepatitis: results of an open-label preliminary trial. Am J Gastroenterol. 1995;90:771–776.
James OFW. Ursodeoxycholic acid treatment for chronic cholestatic liver disease. J Hepatol. 1990;11:5–8.
Calmus Y, Gane P, Rouger P, Poupon R. Hepatic expression of class I and class II major histocompatibility complex molecules in primary biliary cirrhosis: effect of ursodeoxycholic acid. Hepatology. 1990;11:12–15.
Yoshikawa M, Tsujii T, Matsumura K et al. Immunostimulatory effects of ursodeoxycholic acid on immune responses. Hepatology. 1992;16:358–364.
Gianni L, Di Padova F, Zuin M, Podda M. Bile acid-induced inhibition of the lymphoproliferative response to phytohemagglutinin and pokeweed mitogen: an in vitro study. Gastroenterology. 1980;78:231–235.
Hattori Y, Murakami Y, Hattori S, Kuroda H, Kasai K, Shimoda SI. Ursodeoxycholic acid inhibits the induction of nitric oxide synthetase. Eur J Pharmacol. 1996;300:147–150.
Bateson MC. Ursodeoxycholic acid therapy in chronic active hepatitis. Postgrad Med J. 1990;66:781–783.
Crosignani A, Batterzzati PM, Setchell KDR et al. Effects of ursodeoxycholic acid on serum liver enzymes and bile metabolism in chronic active hepatitis: a dose-response study. Hepatology. 1991;13:339–344.
Nakamura K, Yoneda M, Yokohama S et al. Efficacy of ursodeoxycholic acid in Japanese patients with type 1 autoimmune hepatitis. J Gastroenterol Hepatol. 1998;13:490–495.
Czaja AJ, Carpenter HA, Lindor KD. Ursodeoxycholic acid as adjunctive therapy for problematic type 1 autoimmune hepatitis: a randomized placebo-controlled treatment trial. Hepatology. 1999;30:1381–1386.
Clissold SP, Heel RC. Budesonide: a preliminary review of its pharmacodynamic properties and therapeutic efficacy in asthma and rhinitis. Drugs. 1984;28:485–518.
Danielsson A, Prytz H. Oral budesonide for treatment of autoimmune chronic active hepatitis. Aliment Pharmacol Ther. 1994;8:585–590.
Canafax DM, Ascher NL. Cyclosporine immunosuppression. Clin Pharmacol. 1983;2:515–524.
Mistilis SP, Vickers CR, Darroch MH, McCarthy SW. Cyclosporin, a new treatment for autoimmune chronic active hepatitis. Med J Australia. 1985;143:463–465.
Hyams JS, Ballow M, Leichtner AM. Cyclosporine treatment of autoimmune chronic active hepatitis. Gastroenterology. 1987;93:890–893.
Minuk GH. Cyclosporin A in nontransplant-related liver disease. Am J Gastroenterol. 1989;84:1345–1350.
Jackson LD, Song E. Cyclosporin in the treatment of corticosteroid resistant autoimmune chronic active hepatitis. Gut. 1995;36:459–461.
Sherman KE, Narkewicz M, Pinto PC. Cyclosporine in the management of corticosteroid-resistant type 1 autoimmune chronic active hepatitis. J Hepatol. 1994; 21:1040–1047.
Fernandes NF, Redeker AG, Vierling JM, Villamil FG, Fong T-L. Cyclosporine therapy in patients with steroid resistant autoimmune hepatitis. Am J Gastroenterol. 1999;94:241–248.
Lohse AW, Dienes HP, Meyer zum Buschenfelde K-H. Suppression of murine experimental autoimmune hepatitis by T-cell vaccination or immunosuppression. Hepatology. 1998;27:1536–1543.
Schwartz RS. The new immunology-the end of immunosuppressive drug therapy? N Engl J Med. 1999;340:1754–1756.
Lin H, Boiling SF, Linsley PS et al. Long-term acceptance of major histocompatibility complex mismatched cardiac allografts induced by CTLA4Ig plus donor-specific transfusion. J Exp Med. 1993;178:1801–1806.
Guinan EC, Boussiotis VA, Neuberg D et al. Transplantation of anergic histoincompatible bone marrow allografts. N Engl J Med. 1999;340:1704–1714.
Djilali-Saiah I, Schmitz J, Harfouch-Hammond E, Mougenot JF, Bach JF, Caillat-Zucman S. CTLA-4 gene polymorphism is associated with predisposition to coeliac disease. Gut. 1998;43:187–189.
Krokowski M, Bodalski J, Bratek A, Machejko P, Caillat-Zucman S. CTLA-4 gene polymorphism is associated with predisposition to IDDM in a population from central Poland. Diabetes Metab. 1998;24:241–243.
Gonzalez-Escribano MF, Rodriguez R, Valenzuela A, Garcia A, Garcia-Lozano JR, Nunez-Roldan A. CTLA4 polymorphisms in Spanish patients with rheumatoid arthritis. Tissue Antigens. 1999;53:296–300.
Kemp EH, Ajjan RA, Husebye ES et al. A cytotoxic T lymphocyte antigen 4 (CTLA-4) gene polymorphism is associated with autoimmune Addison’s disease in English patients. Clin Endocrinol. 1998;49:609–613.
McCoy KD, Le Gros G. The role of CTLA-4 in the regulation of T cell immune response. Immunol Cell Biol. 1999;77:1–10.
Croxford JL, O’Neill JK, Ali RR et al. Local gene therapy with CTLA4-immunoglobulin fusion protein in experimental allergic encephalomyelitis. Eur J Immunol. 1998;28:3904–3916.
Yu Z, Lennon VA. Mechanism of intravenous immune globulin therapy in antibody-mediated autoimmune diseases. N Engl J Med. 1999;340:227–228.
Carmassi F, Morale R, Puccetti R et al. Efficacy of intravenous immunoglobulin therapy in a case of autoimmune-mediated chronic active hepatitis. Clin Exp Rheumatol. 1992;10:13–17.
Sawada K, Ohnishi K, Kosaka T et al. Exacerbated autoimmune hepatitis successfully treated with leukocytapheresis and bilirubin adsorption therapy. J Gastroenterol. 1997;32:689–695.
Kanzler S, Gerken G, Dienes HP, Meyer zum Buschenfelde KH, Lohse AW. Cyclophosphamide as alternative immunosuppressive therapy for autoimmune hepatitis. Gastroenterology. 1996;110:1228 (abstract).
Slavin S. Treatment of life-threatening autoimmune diseases with myeloablative doses of immunosuppressive agents: experimental background and rationale for ABMT. Bone Marrow Transplant. 1993;12:85–88.
Lee KS, Cottam HB, Houglum K, Wasson DB, Carson D, Chojkier M. Pentoxifylline blocks hepatic stellate cell activation independently of phosphodiesterase inhibitory activity. Am J Physiol. 1997;36:G1094–G1100.
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Czaja, A.J. (2000). Therapy of autoimmune diseases - state of the art. In: Manns, M.P., Paumgartner, G., Leuschner, U. (eds) Immunology and Liver. Falk Symposium, vol 114. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-4000-3_27
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DOI: https://doi.org/10.1007/978-94-011-4000-3_27
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