title: Clinicopathological features of severe and fulminant forms of autoimmune hepatitis.
abstract: OBJECTIVE
  Diagnosis of the acute presentation of autoimmune hepatitis (AIH) is difficult because patients do not always show typical clinicopathological features of AIH. Although some of them progress to fulminant hepatitis, the survival rate of which is <20% without liver transplantation, their clinicopathological features have remained uncertain. We examined them for a better understanding and improvement of the prognosis of "life-threatening" severe and fulminant AIH.
  
  
  METHODS
  Clinical, biochemical and pathological features of 28 patients with severe or fulminant AIH and treatment responses were examined retrospectively.
  
  
  RESULTS
  At the time of admission, mean immunoglobulin G was 2479 ± 1170 mg/dl, with 7 (25%) patients showing normal levels. Anti-nuclear antibody was ≤ 1:40 in 8 (29%). Liver histology showed severe activity in 95% and acute hepatitis in 86% of the patients. Centrilobular necrosis including submassive and massive necrosis was characteristic. Of the 25 patients treated with corticosteroids, 17 responded and 8 did not. Responders to corticosteroids showed younger age and higher prothrombin time (PT) activity than non-responders at the time of corticosteroid administration. The improvement of PT activity during 2 weeks and 4 weeks and total bilirubin level during 4 weeks was statistically significant in responders, but not in non-responders.
  
  
  CONCLUSIONS
  We should diagnose and treat acute onset AIH patients before they develop into severe and fulminant disease. Performing liver biopsy at the early stage of acute onset AIH, evaluating the biopsy specimens precisely and initiating corticosteroid therapy may be essential for improving the prognosis without liver transplantation.
authors.affiliation: Department of Medicine and Clinical Oncology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.
authors.forename: Shin
authors.initials: S
authors.lastname: Yasui
authors.forename: Keiichi
authors.initials: K
authors.lastname: Fujiwara
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Yonemitsu
authors.forename: Shigeto
authors.initials: S
authors.lastname: Oda
authors.forename: Masayuki
authors.initials: M
authors.lastname: Nakano
authors.forename: Osamu
authors.initials: O
authors.lastname: Yokosuka
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000368:Aged; D000410:Alanine Transaminase / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood*; D001663:Bilirubin / Q000097:blood; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008099:Liver / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D011379:Prognosis; D011517:Prothrombin Time; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear; D007074:Immunoglobulin G; D000410:Alanine Transaminase; D001663:Bilirubin
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