Prevalence and Significance of Autoantibodies in Children with Acute Liver Failure.

Objectives: The purpose of this study is to estimate autoantibody (autoAB) frequency, clinical characteristics and 21 day outcome of participants in the Pediatric Acute Liver Failure Study Group (PALFSG) by ANA, SMA and LKM antibody status. Methods: AutoAB 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 one autoAB result was available for 722 (73.2%). At least one autoAB was positive for 202 (28.0%). Diagnoses for autoAB+ subjects were: AIH (63), indeterminate (75) and other (64). AutoAB were more common in Wilson disease (12/32, 37.5%) vs other known diagnoses (52/253, 20.6%, p = 0.03). LKM+ subjects were younger (median 2.4 vs 9.1 yrs, p

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