Incidence and Characteristics of Autoimmune Hepatitis Academic Article uri icon

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abstract

  • BACKGROUND AND OBJECTIVES: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease of unknown etiology, with limited population-based estimates of pediatric incidence. We reported the incidence of pediatric AIH in Canada and described its clinical characteristics. METHODS: We conducted a retrospective cohort study of patients aged <18 years diagnosed with AIH between 2000–2009 at all pediatric centers in Canada. RESULTS: A total of 159 children with AIH (60.3% female, 13.2% type 2 AIH) were identified. Annual incidence was 0.23 per 100000 children. Median age at presentation for type 1 was 12 years (interquartile range: 11–14) versus 10 years for type 2 (interquartile range: 4.5–13) (P = .03). Fatigue (58%), jaundice (54%), and abdominal pain (49%) were the most common presenting symptoms. Serum albumin (33 vs 38 g/L; P = .03) and platelet count (187 000 vs 249 000; P <.001) were significantly lower and the international normalized ratio (1.4 vs 1.2; P <.001) was higher in cirrhotic versus noncirrhotic patients. Initial treatment included corticosteroids (80%), azathioprine (32%), and/or cyclosporine (13%). Response to treatment at 1 year was complete in 90%, and partial in 3%. 3% of patients had no response, and 3% responded and later relapsed. Nine patients underwent liver transplantation, and 4 patients died at a mean follow-up of 4 years. CONCLUSIONS: AIH is uncommon in children and adolescents in Canada. Type 1 AIH was diagnosed 5.5 times more frequently than type 2 AIH. Most patients respond well to conventional therapy, diminishing the need for liver transplantation.

authors

  • Jiménez-Rivera, Carolina
  • Ling, Simon C
  • Ahmed, Najma
  • Yap, Jason
  • Aglipay, Mary
  • Barrowman, Nick
  • Graitson, Samantha
  • Critch, Jeff
  • Rashid, Mohsin
  • Ng, Vicky L
  • Roberts, Eve A
  • Brill, Herbert
  • Dowhaniuk, Jenna
  • Bruce, Garth
  • Bax, Kevin
  • Deneau, Mark
  • Guttman, Orlee R
  • Schreiber, Richard A
  • Martin, Steven
  • Alvarez, Fernando

publication date

  • November 1, 2015

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