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Published on: June 23, 2014
Prevalence and Significance of Autoantibodies in Children With Acute Liver Failure
Michael R Narkewicz1, Simon Horslen, Steven H Belle
1*Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO †Division of Gastroenterology and Hepatology, Seattle Children's Hospital ‡Department of Pediatrics, University of Washington School of Medicine, Seattle, WA §Department of Epidemiology ||Department of Biostatistics, University of Pittsburgh Graduate School of Public Health, Pittsburgh, PA ¶Department of Pediatrics #Department of Developmental Biology, Washington University School of Medicine, St. Louis, MO **Division of Pediatric Gastroenterology, Hepatology/Nutrition and Transplant and Regenerative Medicine Centre, The Hospital for Sick Children ††Department of Pediatrics, University of Toronto, Toronto, Canada ‡‡Division of Pediatric Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children's Hospital §§Department of Pediatrics and Surgery, University of California, San Francisco ||||Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA ¶¶Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia ##Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia ***Department of Pediatrics, University of Pittsburgh School of Medicine †††Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
Insights
Autoantibodies are common in pediatric acute liver failure, found in 28% of patients. While positivity doesn't alter outcomes, liver-kidney microsomal (LKM) antibody-positive children are younger and need more transplants.
Area of Science:
- Pediatric Hepatology
- Autoimmune Diseases
- Clinical Immunology
Background:
- Autoantibodies (auto-ABs) are implicated in various autoimmune conditions.
- Their role in pediatric acute liver failure (PALF) requires further elucidation.
- Understanding auto-AB frequency and clinical associations is crucial for diagnosis and management.
Purpose of the Study:
- To determine the frequency of autoantibodies (antinuclear, smooth muscle, LKM) in children with acute liver failure.
- To analyze the clinical characteristics and 21-day outcomes of these patients based on auto-AB status.
- To investigate the association of auto-ABs with specific diagnoses and treatment responses.
Main Methods:
- Retrospective analysis of 986 participants in the Pediatric Acute Liver Failure Study Group (PALFSG) from 1999-2010.
- Autoantibody testing performed at local and/or central laboratories.
- Subjects categorized into autoimmune hepatitis (AIH), indeterminate, and other diagnosis groups based on auto-AB results.
Main Results:
- Of 722 subjects with available auto-AB results, 202 (28.0%) were positive.
- Auto-ABs were more frequent in Wilson disease (37.5%) than other diagnoses (20.6%, P=0.03).
- Liver-kidney microsomal (LKM)+ subjects were younger (median 2.4 vs 9.1 years) and more likely to receive liver transplants (53.3% vs 31.4%).
Conclusions:
- Autoantibodies are prevalent in pediatric acute liver failure, present in 28% of cases.
- Auto-AB positivity did not significantly impact 21-day outcomes compared to auto-AB negative subjects.
- LKM+ children represent a distinct subgroup requiring further investigation due to unique clinical features and higher transplant rates.
Objectives:
The purpose of the present study is to estimate autoantibody (auto-AB) frequency, clinical characteristics, and 21-day outcome of participants in the Pediatric Acute Liver Failure Study Group (PALFSG) by antinuclear antibody, smooth muscle antibody, and liver-kidney microsomal (LKM) antibody status.
Methods:
Auto-ABs were determined at local and/or central laboratories. Subjects were assigned to autoimmune hepatitis (AIH), indeterminate, and other diagnoses groups.
Results:
Between 1999 and 2010, 986 subjects were enrolled in the PALFSG. At least 1 auto-AB result was available for 722 (73.2%). At least 1 auto-AB was positive for 202 (28.0%). Diagnoses for auto-AB+ subjects were AIH (63), indeterminate (75), and other (64). Auto-ABs were more common in Wilson disease (12/32, 37.5%) compared with other known diagnoses (52/253, 20.6%, P = 0.03). LKM+ subjects were younger (median 2.4 vs 9.1 years, P < 0.001) and more likely to undergo liver transplantation (53.3% vs 31.4% P = 0.02) than other auto-AB+/LKM- subjects. Steroid treatment of subjects who were auto-AB+ was not significantly associated with survival and the subgroup with known diagnoses other than AIH had a higher risk of death.
Conclusions:
Auto-ABs are common in children with acute liver failure, occurring in 28%. Auto-AB+ subjects have similar outcomes to auto-AB negative subjects. LKM+ children are younger and more likely to undergo liver transplantation compared with other auto-AB+ subjects. Although auto-AB may indicate a treatable condition, positivity does not eliminate the need for a complete diagnostic evaluation because auto-ABs are present in other conditions. The significance of auto-AB in pediatric acute liver failure remains uncertain, but LKM+ appears to identify a unique population of children who merit further study.
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