Incidence and Characteristics of Autoimmune Hepatitis

Author:

Jiménez-Rivera Carolina1,Ling Simon C.2,Ahmed Najma3,Yap Jason4,Aglipay Mary1,Barrowman Nick1,Graitson Samantha1,Critch Jeff5,Rashid Mohsin6,Ng Vicky L.2,Roberts Eve A.2,Brill Herbert7,Dowhaniuk Jenna K.7,Bruce Garth8,Bax Kevin9,Deneau Mark10,Guttman Orlee R.11,Schreiber Richard A.11,Martin Steven12,Alvarez Fernando13

Affiliation:

1. University of Ottawa and Children’s Hospital of Eastern Ontario, Ottawa, Ontario, Canada;

2. University of Toronto and The Hospital for Sick Children, Toronto, Ontario, Canada;

3. McGill University and Montreal Children’s Hospital, Montreal, Quebec, Canada;

4. University of Alberta and Stollery Children’s Hospital, Edmonton, Alberta, Canada;

5. Memorial University of Newfoundland and Janeway Children’s Hospital, St. John's, Newfoundland, Canada;

6. Dalhousie University and IWK Health Center, Halifax, Nova Scotia, Canada;

7. McMaster University and McMaster Children’s Hospital, Hamilton, Ontario, Canada;

8. University of Saskatchewan and Children’s Hospital of Saskatchewan, Saskatoon, Saskatchewan, Canada;

9. University of Western Ontario and London Health Science Center, London, Ontario, Canada;

10. University of Manitoba and The Children’s Hospital of Winnipeg, Winnipeg, Manitoba, Canada;

11. University of British Columbia and BC Children’s Hospital, Vancouver, British Columbia, Canada;

12. University of Calgary and Alberta Children’s Hospital Calgary, Alberta, Canada; and

13. University of Montreal and Centre Hospitalier Universitaire Sainte-Justine, Montreal, Quebec, Canada

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.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology and Child Health

Reference46 articles.

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