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Published on: May 29, 2020
Characteristics and outcome of autoimmune liver disease in Asian children
Way S Lee1, Su H Lum, Chooi B Lim
1Department of Paediatrics, University Malaya Medical Center, 59100, Kuala Lumpur, Malaysia, leews@um.edu.my.
Insights
This study highlights that delayed diagnosis and treatment worsen outcomes for children with autoimmune liver disease (AILD) in Malaysia, despite common remission with medication. Early intervention is crucial for better survival rates in pediatric AILD patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Autoimmune Diseases
Background:
- Autoimmune liver disease (AILD) in Asian children is poorly understood.
- This study investigates the clinical characteristics and prognostic factors of AILD in a Malaysian pediatric population.
Purpose of the Study:
- To describe the clinical features of autoimmune liver disease in Asian children.
- To identify predictors of outcome in childhood autoimmune liver disease.
Main Methods:
- Retrospective review of pediatric autoimmune liver disease cases (autoimmune hepatitis types 1 and 2, autoimmune sclerosing cholangitis) from two Malaysian liver units.
- Analysis of clinical data, treatment responses, and patient outcomes.
Main Results:
- Of 32 children with AILD, 56% had cirrhosis at presentation. Overall survival was 78%, with 75% surviving with a native liver.
- Remission was achieved in 66% of patients with immunosuppressive therapy (prednisolone, azathioprine).
- Delayed diagnosis and treatment significantly worsened patient outcomes (p=0.012).
Conclusions:
- While medical therapy can induce remission in most childhood AILD cases, timely diagnosis and treatment are critical for improving outcomes.
- Delay in seeking care adversely impacts the prognosis of autoimmune liver disease in Malaysian children.
Background:
Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population.
Methods:
Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia.
Results:
At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [survival with native liver vs. LT or death (duration between onset of disease and treatment; median ± standard error) = 2.5 ± 2.9 months vs. 24.0 ± 13.3 months; p = 0.012].
Conclusions:
Although remission was achieved in the majority of patients with prednisolone and/or azathioprine therapy, delay in seeking diagnosis and treatment adversely affects the outcome of childhood AILD in Malaysia.
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