title: Natural History and Treatment Outcomes of Severe Autoimmune Hepatitis.
abstract: OBJECTIVE
  The aim of this study was to analyze the natural history and treatment outcomes of autoimmune hepatitis (AIH) variants presenting with severe-AIH.
  
  
  BACKGROUND
  Severe acute presentation is an uncommon manifestation of AIH, and it remains poorly characterized.
  
  
  METHODS
  We included 101 patients with AIH from January 2011 to December 2015. Patients were classified as seropositive-AIH and seronegative-AIH. Patients with acute liver failure, acute-on-chronic liver failure, and severe acute hepatitis were defined as severe-AIH patients. Patient characteristics and treatment outcomes with follow-up until 12 months were analyzed between the different groups.
  
  
  RESULTS
  Out of 101 cases, 24 (23.76%) had severe AIH. Of them 9 (37.5%) had severe acute hepatitis, 3 (12.5%) had acute liver failure, and 12 (50%) had acute-on-chronic liver failure. Seronegative-AIH patients presented with severe-AIH significantly more frequently compared with seropositive-AIH patients (50% vs. 20.27%, P=0.022). Severe-AIH had 50% complete responders, 25% partial responders, and 25% treatment failures. Jaundice (88.88% vs. 68.7%, P=0.048), encephalopathy (55.55% vs. 6.66%, P=0.014), and higher international normalized ratio values (2.17±0.60 vs. 1.82±0.14, P=0.038) were factors associated with nonresponse rather than the presence or absence of autoantibodies in severe-AIH. The hazard ratio for predicting remission in the non-severe AIH group as compared with the severe-AIH group was 1.502, which was statistically not significant (95% CI, 0.799-2.827; P=0.205).
  
  
  CONCLUSIONS
  Approximately 24% of patients with AIH have severe-AIH. Conventional autoantibodies are often absent in severe-AIH; however, it does not alter the outcome. Immunosuppressants should be given expediently in patients with severe-AIH.
authors.affiliation: Department of Gastroenterology, Topiwala National Medical College and BYL Ch Hospital, Mumbai, Maharashtra, India.
authors.forename: Nikhil
authors.initials: N
authors.lastname: Sonthalia
authors.forename: Pravin M
authors.initials: PM
authors.lastname: Rathi
authors.forename: Samit S
authors.initials: SS
authors.lastname: Jain
authors.forename: Ravindra G
authors.initials: RG
authors.lastname: Surude
authors.forename: Ashok R
authors.initials: AR
authors.lastname: Mohite
authors.forename: Sunil V
authors.initials: SV
authors.lastname: Pawar
authors.forename: Qais
authors.initials: Q
authors.lastname: Contractor
mesh_terms: D000208:Acute Disease; D065290:Acute-On-Chronic Liver Failure / Q000175:diagnosis* / Q000276:immunology; D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000276:immunology*; D002648:Child; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017114:Liver Failure, Acute / Q000175:diagnosis* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D012074:Remission Induction; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents