Original article—liver, pancreas, and biliary tractValidation and Modification of Simplified Diagnostic Criteria for Autoimmune Hepatitis in Children
Section snippets
Study Population
All children (age at presentation, <21 y) included in this study were from a single pediatric hepatology center at a tertiary care hospital. Children with AIH were identified through International Classification of Diseases, 9th revision codes, pathology reports, and cross-referencing with pediatric hepatology patient lists from 1991 to 2010. AIH subjects had to have all baseline laboratory, histology, demographic, and clinical information necessary to calculate and confirm the diagnosis of
Results
An initial 238 subjects with various liver diseases were evaluated. We identified 77 patients (37 AIH, 40 non-AIH) who had complete data available to calculate the 1999 score (Figure 1). Within the AIH group, 31 of 37 subjects had IgG levels to calculate the 2008 score but all 37 subjects had either IgG or globulin levels, or both, available at diagnosis. Of these patients, 36 had AIH and 1 had overlap AIH-PSC at the time of diagnosis. From the non-AIH group, 26 of 40 subjects had IgG levels to
Discussion
This cross-sectional study compared the 2008 criteria for diagnosing AIH with the 1999 revised original criteria in a pediatric cohort with liver disease. We showed that the 2008 simplified criteria are useful for pediatric patients. Prior studies evaluated these criteria compared with codified descriptive criteria first developed by the IAIHG in 1993.2, 3, 4, 5, 6 Although all of our AIH patients met the diagnostic criteria based on the 1993 criteria, we chose to use the 1999 original revised
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Cited by (67)
Autoimmune Hepatitis
2023, MacSween's Pathology of the Liver, Eighth EditionRecent Insights into Pediatric Primary Sclerosing Cholangitis
2022, Clinics in Liver DiseaseAcute onset of autoimmune hepatitis in children and adolescents
2020, Hepatobiliary and Pancreatic Diseases InternationalCitation Excerpt :The number of patients is relatively small and they were recruited from a single-center. IAIHG score was used for patient enrollment at the beginning of study; however, if validated simplified diagnostic criteria [24] were used, number of diagnosed AI-ALF cases may have been higher, as was shown both in children and adult patients [24,25]. In conclusion, up to one half of pediatric AIH cases manifest as acute hepatitis and their diagnosis may be difficult - predominantly in patients with fulminant disease course, where not all of the diagnostic criteria are necessarily met.
Development and validation of a new simplified diagnostic scoring system for pediatric autoimmune hepatitis
2019, Digestive and Liver DiseaseCitation Excerpt :To overcome these difficulties, the IAIHG proposed a simplified scoring system in 2008, that takes into account only the presence of autoantibodies, IgG levels, histopathology and absence of viral markers [8,9]. Some validation studies have been carried out on the accuracy of simplified criteria, showing that they are not perfectly suitable to the juvenile form of the disease, even further in cases with ALF onset [10–18]. Furthermore, children with AIH often exhibit lower autoantibodies titers than adults, are less prone to hypergammaglobulinemia, and autoimmune sclerosing cholangitis (AISC) require exclusion as it can easily be mistaken for AIH [17,19,20].
Conflicts of interest The authors disclose no conflicts.
Funding Supported by a National Institutes of Health T32 grant (DK007762) and a University of California San Francisco Liver Center grant (P30 DK26743).