Sakulsaengprapha Vorada, Wasuwanich Paul, Naraparaju Gayathri, Korotkaya Yelena, Thawillarp Supharerk, Oshima Kiyoko, Karwowski Christine, Scheimann Ann O, Karnsakul Wikrom
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
College of Medicine, University of Florida, Gainesville, FL 32610, USA.
Biology (Basel). 2023 Mar 21;12(3):479. doi: 10.3390/biology12030479.
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.
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.
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.
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.
许多肝脏疾病酷似自身免疫性肝炎（AIH）。研究人员开发了国际自身免疫性肝炎小组（IAIHG）评分系统，以弥补AIH缺乏特异性诊断测试的不足。该评分系统在设计时未考虑儿科患者，因此其在儿科的应用存在局限性。此外，关于肝活检结合该评分系统使用的价值的信息有限。
在这项回顾性研究中，我们评估了肝活检评分对0至18岁疑似AIH患者的IAIHG评分系统的影响。我们还分析了与最终AIH诊断相关的人口统计学数据和实验室值。
我们发现活检期间发现的界面性肝炎和主要浆细胞与最终的AIH诊断显著相关。我们还发现异常实验室值与AIH诊断相关。我们发现肝活检后计算的IAIHG评分在受试者操作特征曲线（AUROC）下显示出更大的面积，为0.95，而肝活检前计算的评分为0.88。纳入活检指标降低了优化的临界值分数和测试特异性。
纳入肝脏组织病理学特征可提高IAIHG评分系统的性能。进一步研究以确定肝脏组织学中的其他潜在因素可能会改善IAIHG测试在儿科人群中的性能指标。
