Applicability of International Autoimmune Hepatitis Group (IAIHG) Scoring System for Autoimmune Hepatitis in Pediatrics
Vorada Sakulsaengprapha,
Paul Wasuwanich,
Gayathri Naraparaju,
Yelena Korotkaya,
Supharerk Thawillarp,
Kiyoko Oshima,
Christine Karwowski,
Ann O. Scheimann,
Wikrom Karnsakul
Affiliations
Vorada Sakulsaengprapha
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Paul Wasuwanich
College of Medicine, University of Florida, Gainesville, FL 32610, USA
Gayathri Naraparaju
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Yelena Korotkaya
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Supharerk Thawillarp
Department of Health Policy and Management, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21205, USA
Kiyoko Oshima
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Christine Karwowski
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Ann O. Scheimann
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Wikrom Karnsakul
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
Introduction: Many hepatologic pathologies mimic autoimmune hepatitis (AIH). Researchers developed the International Autoimmune Hepatitis Group (IAIHG) scoring system to compensate for the lack of specific diagnostic tests for AIH. The scoring system was not designed with pediatric patients in mind, so there are limits to its pediatric use. Additionally, there is limited information on the value of a liver biopsy in conjunction with its use. Methods: In this retrospective study, we evaluated the effect of liver biopsy scores on the IAIHG scoring system in patients that were 0–18 years old with suspected AIH. We also analyzed demographic data and laboratory values associated with a final AIH diagnosis. Results: We found that interface hepatitis and predominant plasma cells found during the biopsy were significantly associated with a final AIH diagnosis. We also found that abnormal laboratory values were associated with an AIH diagnosis. We found that IAIHG scores calculated post-liver biopsy showed a greater area under the receiver operating characteristic curve (AUROC) of 0.95, which was compared to 0.88 for the scores calculated before a liver biopsy. Including biopsy metrics lowered the optimized cutoff score and test specificity. Conclusion: Incorporating liver histopathological features improved the performance of the IAIHG scoring system. Further studies to identify other potential elements in liver histology may improve the performance metrics of the IAIHG test in the pediatric population.
