{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T18:18:02Z","timestamp":1767205082868,"version":"build-2238731810"},"reference-count":28,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,3,21]],"date-time":"2023-03-21T00:00:00Z","timestamp":1679356800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.mdpi.com"],"crossmark-restriction":true},"short-container-title":["Biology"],"abstract":"<jats:p>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\u201318 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.<\/jats:p>","DOI":"10.3390\/biology12030479","type":"journal-article","created":{"date-parts":[[2023,3,21]],"date-time":"2023-03-21T03:15:03Z","timestamp":1679368503000},"page":"479","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Applicability of International Autoimmune Hepatitis Group (IAIHG) Scoring System for Autoimmune Hepatitis in Pediatrics"],"prefix":"10.3390","volume":"12","author":[{"given":"Vorada","family":"Sakulsaengprapha","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7555-453X","authenticated-orcid":false,"given":"Paul","family":"Wasuwanich","sequence":"additional","affiliation":[{"name":"College of Medicine, University of Florida, Gainesville, FL 32610, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gayathri","family":"Naraparaju","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yelena","family":"Korotkaya","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Supharerk","family":"Thawillarp","sequence":"additional","affiliation":[{"name":"Department of Health Policy and Management, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyoko","family":"Oshima","sequence":"additional","affiliation":[{"name":"Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine","family":"Karwowski","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ann","family":"Scheimann","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7288-5459","authenticated-orcid":false,"given":"Wikrom","family":"Karnsakul","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"998","DOI":"10.1002\/hep.1840180435","article-title":"Meeting report: International autoimmune hepatitis group","volume":"18","author":"Johnson","year":"1993","journal-title":"Hepatology"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0168-8278(00)80004-5","article-title":"Overlap of autoimmune hepatitis and primary sclerosing cholangitis: An evaluation of a modified scoring system","volume":"33","author":"Kaya","year":"2000","journal-title":"J. Hepatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1008","DOI":"10.2169\/internalmedicine.39.1008","article-title":"Re-analysis of clinical features of 89 patients with autoimmune hepatitis using the revised scoring system proposed by the International Autoimmune Hepatitis Group","volume":"39","author":"Omagari","year":"2000","journal-title":"Intern. Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1016\/S0168-8278(99)80297-9","article-title":"International Autoimmune Hepatitis Group Report: Review of criteria for diagnosis of autoimmune hepatitis","volume":"31","author":"Alvarez","year":"1999","journal-title":"J. Hepatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1016\/S1542-3565(04)00396-9","article-title":"Diagnosing autoimmune hepatitis in children: Is the International Autoimmune Hepatitis Group scoring system useful?","volume":"2","author":"Ebbeson","year":"2004","journal-title":"Clin. Gastroenterol. Hepatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1002\/jgh3.12046","article-title":"Non-alcoholic fatty liver disease in patients with autoimmune hepatitis","volume":"2","author":"Takahashi","year":"2018","journal-title":"JGH Open."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"71","DOI":"10.5858\/2000-124-0071-GFCUOT","article-title":"Guidelines for clinical use of the antinuclear antibody test and tests for specific autoantibodies to nuclear antigens. American College of Pathologists","volume":"124","author":"Kavanaugh","year":"2000","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1782","DOI":"10.1002\/hep.22825","article-title":"Validation of simplified diagnostic criteria for autoimmune hepatitis in italian patients","volume":"49","author":"Muratori","year":"2009","journal-title":"Hepatology"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"e81","DOI":"10.3928\/19382359-20180126-01","article-title":"Autoimmune Hepatitis in Children","volume":"47","author":"Pathak","year":"2018","journal-title":"Pediatr. Ann."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"18017","DOI":"10.1038\/nrdp.2018.17","article-title":"Autoimmune hepatitis","volume":"4","author":"Vergani","year":"2018","journal-title":"Nat. Rev. Dis. Prim."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1053\/jhep.2002.34944","article-title":"Diagnosis and treatment of autoimmune hepatitis","volume":"36","author":"Czaja","year":"2002","journal-title":"Hepatology"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2117","DOI":"10.1002\/hep.23577","article-title":"Causality assessment in drug-induced liver injury using a structured expert opinion process: Comparison to the Roussel-Uclaf causality assessment method","volume":"51","author":"Rockey","year":"2010","journal-title":"Hepatology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.jhep.2010.12.012","article-title":"Autoimmune hepatitis","volume":"55","author":"Lohse","year":"2011","journal-title":"J. Hepatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1273","DOI":"10.1111\/j.1365-2036.2005.02488.x","article-title":"Clinical features of type 1 autoimmune hepatitis in elderly Italian patients","volume":"21","author":"Granito","year":"2005","journal-title":"Aliment. Pharmacol. Ther."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1002\/hep.31065","article-title":"Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases","volume":"72","author":"Mack","year":"2020","journal-title":"Hepatology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1007\/s10620-006-9495-4","article-title":"Evidence of a genetic basis for the different geographic occurrences of liver\/kidney microsomal antibody type 1 in hepatitis C","volume":"52","author":"Muratori","year":"2007","journal-title":"Dig. Dis. Sci."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1097\/MPG.0000000000001801","article-title":"Diagnosis and Management of Pediatric Autoimmune Liver Disease: ESPGHAN Hepatology Committee Position Statement","volume":"66","author":"Vergani","year":"2018","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_18","first-page":"10","article-title":"Autoimmune liver disease\u2014Are there spectra that we do not know?","volume":"12","author":"Fallatah","year":"2011","journal-title":"Comp. Hepatol."},{"key":"ref_19","unstructured":"Lala, V., Zubair, M., and Minter, D.A. (2022). Liver Function Tests. StatPearls, StatPearls Publishing."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1111\/j.1572-0241.2007.01153.x","article-title":"Improving the end point of corticosteroid therapy in type 1 autoimmune hepatitis to reduce the frequency of relapse","volume":"102","author":"Carpenter","year":"2007","journal-title":"Am. J. Gastroenterol."},{"key":"ref_21","doi-asserted-by":"crossref","unstructured":"Vuppalanchi, R., and Chalasani, N. (2011). 5\u2013Laboratory tests in liver disease. Practical Hepatic Pathology: A Diagnostic Approach, Sanders (Elsevier).","DOI":"10.1016\/B978-0-443-06803-4.00005-8"},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Danan, G., and Teschke, R. (2015). RUCAM in Drug and Herb Induced Liver Injury: The Update. Int. J. Mol. Sci., 17.","DOI":"10.3390\/ijms17010014"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1961","DOI":"10.1053\/j.gastro.2008.08.050","article-title":"Clinical Correlates of Histopathology in Pediatric Nonalcoholic Steatohepatitis","volume":"135","author":"Patton","year":"2008","journal-title":"Gastroenterology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e238400","DOI":"10.1136\/bcr-2020-238400","article-title":"Non-alcoholic steatohepatitis or autoimmune hepatitis? Sometimes a closer look under the surface is needed","volume":"13","author":"Dalekos","year":"2020","journal-title":"BMJ Case Rep."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e12696","DOI":"10.1111\/ijpo.12696","article-title":"Significance of autoantibody seropositivity in children with obesity and non-alcoholic fatty liver disease","volume":"16","author":"Yodoshi","year":"2021","journal-title":"Pediatr. Obes."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1002\/hep.22322","article-title":"Simplified criteria for the diagnosis of autoimmune hepatitis","volume":"48","author":"Hennes","year":"2008","journal-title":"Hepatology"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1007\/s12016-014-8432-0","article-title":"Emperipolesis mediated by CD8 T cells is a characteristic histopathologic feature of autoimmune hepatitis","volume":"48","author":"Miao","year":"2015","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1862","DOI":"10.1002\/hep.32591","article-title":"Noninvasive biomarkers for the diagnosis and management of autoimmune hepatitis","volume":"76","author":"Harrington","year":"2022","journal-title":"Hepatology"}],"updated-by":[{"DOI":"10.3390\/biology12050727","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2023,3,21]],"date-time":"2023-03-21T00:00:00Z","timestamp":1679356800000}}],"container-title":["Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2079-7737\/12\/3\/479\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,3]],"date-time":"2025-08-03T14:27:30Z","timestamp":1754231250000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2079-7737\/12\/3\/479"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,21]]},"references-count":28,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,3]]}},"alternative-id":["biology12030479"],"URL":"https:\/\/doi.org\/10.3390\/biology12030479","relation":{},"ISSN":["2079-7737"],"issn-type":[{"value":"2079-7737","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,21]]}}}