Incidence and Characteristics of Autoimmune Hepatitis

• Published in: Pediatrics (Evanston) Vol. 136; no. 5; p. e1237
• Main 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 K, Bruce, Garth, Bax, Kevin, Deneau, Mark, Guttman, Orlee R, Schreiber, Richard A, Martin, Steven, Alvarez, Fernando
• Format: Journal Article
• Language: English
• Published: United States 01.11.2015
• Subjects: Adolescent; Adrenal Cortex Hormones - therapeutic use; Azathioprine - therapeutic use; Canada - epidemiology; Child; Cholangiopancreatography, Magnetic Resonance; Cyclosporine - therapeutic use; Female; Hepatitis, Autoimmune - diagnosis; Hepatitis, Autoimmune - epidemiology; Hepatitis, Autoimmune - mortality; Hepatitis, Autoimmune - surgery; Hepatitis, Autoimmune - therapy; Humans; Immunosuppressive Agents - therapeutic use; Incidence; Liver Cirrhosis - epidemiology; Liver Transplantation; Male; Retrospective Studies; Survival Analysis; Treatment Outcome; Canada
• DOI: 10.1542/peds.2015-0578

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. We conducted a retrospective cohort study of patients aged <18 years diagnosed with AIH between 2000-2009 at all pediatric centers in Canada. 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. 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.