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Published on: May 29, 2020
Incidence and Characteristics of Autoimmune Hepatitis
Carolina Jiménez-Rivera1, Simon C Ling2, Najma Ahmed3
1University of Ottawa and Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada; cajimenez@cheo.on.ca.
Insights
Autoimmune hepatitis (AIH) is rare in Canadian children, with Type 1 AIH being more common than Type 2. Most pediatric patients with AIH respond well to standard treatments, reducing the need for liver transplants.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Immunology
Background:
- Autoimmune hepatitis (AIH) is a progressive, inflammatory liver disease with unknown causes.
- Population-based incidence data for pediatric AIH is limited.
- Understanding pediatric AIH epidemiology is crucial for early diagnosis and management.
Purpose of the Study:
- To determine the incidence of pediatric autoimmune hepatitis in Canada.
- To describe the clinical characteristics and treatment outcomes of pediatric AIH.
- To compare the incidence and presentation of Type 1 vs. Type 2 AIH in children.
Main Methods:
- Retrospective cohort study of patients under 18 years old diagnosed with AIH.
- Data collected from all Canadian pediatric centers between 2000 and 2009.
- Analysis included incidence rates, clinical features, laboratory values, treatment responses, and outcomes.
Main Results:
- 159 pediatric AIH cases identified; annual incidence of 0.23 per 100,000 children.
- Type 1 AIH was diagnosed 5.5 times more frequently than Type 2 AIH.
- Common symptoms included fatigue, jaundice, and abdominal pain; cirrhotic patients had lower albumin and platelets, and higher INR.
Conclusions:
- Autoimmune hepatitis is uncommon in Canadian children and adolescents.
- Most pediatric AIH patients respond well to conventional therapies like corticosteroids and azathioprine.
- Effective treatment of AIH in children can decrease the necessity for liver transplantation.
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.
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