Original article—liver, pancreas, and biliary tract
Validation and Modification of Simplified Diagnostic Criteria for Autoimmune Hepatitis in Children

https://doi.org/10.1016/j.cgh.2011.11.030Get rights and content

Background & Aims

Criteria for the diagnosis of autoimmune hepatitis (AIH) were formalized in 1993 and revised in 1999. Simplified criteria were developed in 2008 for adults only. We aimed to establish clinically useful diagnostic criteria for AIH in children by validating the 2008 criteria in a pediatric cohort.

Methods

Baseline data were available in 37 and 31 AIH and 40 and 26 non-AIH subjects to calculate 1999 and 2008 criteria, respectively. Sensitivity and specificity of the simplified criteria were calculated using 1999 criteria as the standard for subjects with available data for both criteria.

Results

The 1999 standard designated 29 of 31 subjects (94%) as definite AIH and 2 of 31 subjects (6%) as probable AIH. The simplified criteria identified 25 of 31 subjects (81%) as definite AIH, 2 of 31 subjects (6%) as probable AIH. Only 1 of 5 patients with AIH who presented with fulminant hepatic failure (FHF) was identified by the simplified criteria as having AIH. The 2008 diagnostic criteria had a sensitivity of 87% and a specificity of 89% (area under the receiver operating characteristic curve, 0.98). After removing data from patients with FHF from the analysis, the sensitivity increased to 100%. Modifying the 2008 diagnostic criteria to include either level of globulin or immunoglobulin G resulted in a similar sensitivity (92%) and specificity (95%) values (area under the receiver operating characteristic curve, 0.99).

Conclusions

The 2008 criteria diagnose AIH in children with high levels of sensitivity and specificity, and are easier to use in the clinic. Diagnosis of AIH in patients who present in FHF requires the 1999 criteria. Levels of globulin and immunoglobulin G can be used interchangeably in the simplified diagnostic criteria.

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

Cited by (67)

  • Autoimmune Hepatitis

    2023, MacSween's Pathology of the Liver, Eighth Edition
  • Acute onset of autoimmune hepatitis in children and adolescents

    2020, Hepatobiliary and Pancreatic Diseases International
    Citation 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 Disease
    Citation 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].

View all citing articles on Scopus

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).

View full text