Abstract
Background
The diagnosis of acute-onset autoimmune hepatitis (AIH) has been difficult because patients do not always show clinicopathological features typical of AIH. We examined the important requirements for a definitive diagnosis of acute-onset AIH.
Methods
Clinical, biochemical, and pathological features of 18 acute-onset AIH patients (16 women, 2 men; mean age, 54.3 ± 12.3 years) with no history of liver disease and no signs of chronicity were examined.
Results
Mean ALT was 679 ± 431 IU/l, and mean T-Bil was 2.4 ± 2.9 mg/dl. Mean IgG was 1801 ± 446 mg/dl, with 7 patients (39%) showing normal levels. Antinuclear antibody was ≤1: 40 in 7 (39%). Liver histology showed severe activity in 17 (94%) of the patients and severe acute hepatitis in 7 (39%). Centrizonal necrosis and plasma cell accumulation were characteristic for acute-onset AIH. AIH score ranged from 7 to 18 (13.2 ± 3.8) before treatment. All patients were diagnosed and treated early and responded completely to therapy.
Conclusions
Histological examination of the liver is necessary for early diagnosis of acute-onset AIH. Moreover, we should evaluate liver biopsy specimens precisely and should be ready for a timely initiation of corticosteroid therapy to improve the prognosis.
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References
Czaja AJ. Autoimmune hepatitis: evolving concepts and treatment strategies. Dig Dis Sci 1995;40:435–456.
Czaja AJ, Carpenter HA. Sensitivity, specificity and predictability of biopsy interpretations in chronic hepatitis. Gastroenterology 1993;105:1824–1832.
Soloway RD, Summerskill WHJ, Baggenstoss AH, Geall MG, Gitnick GL, Elveback LR, 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.
Mistilis SP, Skyring AP, Blackburn CRB. Natural history of active chronic hepatitis. I. Clinical features, course, diagnostic criteria, morbidity, mortality, and survival. Australas Ann Med 1968;17:214–223.
Czaja AJ, Davis GL, Ludwig J, Baggenstoss AH, Taswell HF. Autoimmune features as determinants of prognosis in steroid-treated chronic active hepatitis of uncertain etiology. Gastroenterology 1983;85:713–717.
Crapper RM, Bhathal PS, Mackay IR, Frazer IH. “Acute” autoimmune hepatitis. Digestion 1986;34:216–225.
Amontree JS, Stuart TD, Bredfeldt JE. Autoimmune chronic active hepatitis masquerading as acute hepatitis. J Clin Gastroenterol 1989;11:303–307.
Nikias GA, Batts KP, Czaja AJ. The nature and prognostic implications of autoimmune hepatitis with an acute presentation. J Hepatol 1994;21:866–871.
Porta G, Da Costa Gayotto LC, Alvarez F. Anti-liver-kidney microsome antibody-positive autoimmune hepatitis presenting as fulminant liver failure. J Pediatr Gastroenterol Nutr 1990;11:138–140.
Alvarez F, Berg PA, Bianchi FB, Bianchi L, Burroughs AK, Cancado EL, Chapman RW, et al. International Autoimmune Hepatitis Group report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999;31:929–938.
Desmet VJ, Gerber M, Hoofnagle JH, Manns M, Scheuer PJ. Classification of chronic hepatitis: diagnosis, grading and staging. Hepatology 1994;19:1513–15120.
Maria VAJ, Victorino RMM. Development and validation of a clinical scale for the diagnosis of drug-induced hepatitis. Hepatology 1997;26:664–669.
Abe M, Onji M, Kawai-Ninomiya K, Michitaka K, Matsuura B, Hiasa Y, et al. Clinicopathologic features of the severe form of acute type 1 autoimmune hepatitis. Clin Gastroenterol Hepatol 2007;5:255–258.
Abe M, Hiasa Y, Masumoto T, Kumagi T, Akbar SM, Ninomiya T, et al. Clinical characteristics of autoimmune hepatitis with histological feature of acute hepatitis. Hepatol Res 2001;21:213–219.
Ferrari R, Pappas G, Agostinelli D, Muratori P, Muratori L, Lenzi M, et al. Type 1 autoimmune hepatitis: patterns of clinical presentation and differential diagnosis of the “acute” type. Q J Med 2004;97:407–412.
Burgart LJ, Batts KP, Ludwig J, Nikias GA, Czaja AJ. Recent onset autoimmune hepatitis. Biopsy findings and clinical correlations. Am J Surg Pathol 1995;19:699–708.
Kaymakoglu S, Cakaloglu Y, Demir K, Turkoglu S, Badur S, Gurel S, et al. Is severe cryptogenic chronic hepatitis similar to autoimmune hepatitis? J Hepatol 1998;28:78–83.
Potthoff A, Deterding K, Trautwein C, Flemming P, Strassburg CP, Manns MP, et al. Steroid treatment for severe acute cryptogenic hepatitis. Z Gastroenterol 2007;45:159.
Lefkowitch JH, Apfelbaum TF, Weinberg L, Forester G. Acute liver biopsy lesions in early autoimmune (“lupoid”) chronic active hepatitis. Liver 1984;4:379–386.
Ohta Y, Onji M. Clinical characteristics of autoimmune hepatitis in Japan. In: Nishioka M, Toda G, Zeniya M, editors. Autoimmune hepatitis. Amsterdam: Elsevier; 1993. p. 45–53.
Singh R, Nair S, Farr G, Mason A, Perrillo R. Acute autoimmune hepatitis presenting with centrizonal liver disease: case report and review of the literature. Am J Gastroenterol 2002;97:2670–2673.
Hofer H, Oesterreicher C, Wrba F, Ferenci P, Penner E. Centrilobular necrosis in autoimmune hepatitis: a histological feature associated with acute clinical presentation. J Clin Pathol 2006;59:246–249.
Lohse AW, Manns M, Dienes HP, Meyer zum Buschenfelde KH, Cohen IR. Experimental autoimmune hepatitis: disease induction, time course and T-cell reactivity. Hepatology 1990;11:24–30.
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Fujiwara, K., Fukuda, Y. & Yokosuka, O. Precise histological evaluation of liver biopsy specimen is indispensable for diagnosis and treatment of acute-onset autoimmune hepatitis. J Gastroenterol 43, 951–958 (2008). https://doi.org/10.1007/s00535-008-2254-x
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DOI: https://doi.org/10.1007/s00535-008-2254-x