title: Long-term outcome of Japanese patients with type 1 autoimmune hepatitis.
abstract: The long-term outcome of patients with autoimmune hepatitis (AIH) in Japan has not been well-defined. The aim of this study was to clarify the outcome of this disease over a long follow-up period compared with that of the general Japanese population as well as that among patients. A total of 203 AIH patients were enrolled for a mean follow-up period of 131 months. All patients were treated with corticosteroids with or without azathioprine. The overall survival of AIH patients was similar to that of the general population in Japan. The prognosis of AIH subgroups divided according to disease severity, sex, incidence of relapse, liver histology, presence of cirrhosis, probable or definite AIH score, antibody to hepatitis B core antigen antibody positivity, or human leukocyte antigen DR4-positivity did not differ greatly among patients. However, the prognosis of patients experiencing two or more relapses was significantly poorer than that of patients with remission or a single relapse both in univariate (P < 0.001) and multivariate (P = 0.020) analyses. The development of liver malignancy was also a possibility among AIH patients with multiple relapses. Severe adverse effects of corticosteroids were rare, even in patients who underwent long-term treatment.
  
  
  CONCLUSIONS
  Repeated relapses of AIH are significantly associated with a poorer long-term prognosis in Japan. AIH patients can expect a similar prognosis to that of the general population, provided they are adequately managed with continuous low doses of immunosuppressive therapy, especially after the first relapse.
authors.affiliation: Department of Medicine, Shinshu University School of Medicine, Matsumoto, Japan. kanamey@shinshu-u.ac.jp
authors.forename: Kaname
authors.initials: K
authors.lastname: Yoshizawa
authors.forename: Akihiro
authors.initials: A
authors.lastname: Matsumoto
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Ichijo
authors.forename: Takeji
authors.initials: T
authors.lastname: Umemura
authors.forename: Satoru
authors.initials: S
authors.lastname: Joshita
authors.forename: Michiharu
authors.initials: M
authors.lastname: Komatsu
authors.forename: Naoki
authors.initials: N
authors.lastname: Tanaka
authors.forename: Eiji
authors.initials: E
authors.lastname: Tanaka
authors.forename: Masao
authors.initials: M
authors.lastname: Ota
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Katsuyama
authors.forename: Kendo
authors.initials: K
authors.lastname: Kiyosawa
authors.forename: Masanori
authors.initials: M
authors.lastname: Abe
authors.forename: Morikazu
authors.initials: M
authors.lastname: Onji
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001172:Arthritis, Rheumatoid / Q000401:mortality; D044466:Asian People / Q000706:statistics & numerical data*; D002543:Cerebral Hemorrhage / Q000401:mortality; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage; D019693:Hepatitis, Autoimmune / Q000188:drug therapy / Q000208:ethnology* / Q000401:mortality*; D006801:Humans; D007564:Japan / Q000453:epidemiology; D017093:Liver Failure / Q000401:mortality; D008113:Liver Neoplasms / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D011446:Prospective Studies; D012008:Recurrence; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone