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Abstract：
UNASSIGNED: Children with autoimmune hepatitis (AIH) often present with symptoms similar to those of other liver diseases. This study consists of a comparison between the clinical and histological characteristics of AIH and those of other four AIH-like liver diseases [i.e., drug-induced liver injury (DILI), gene deficiency, infectious liver disease and other etiology of liver disease], as well as an evaluation of the AIH scoring system\'s diagnostic performance.
UNASSIGNED: All children with AIH-like liver disease at our center from January 2013 to December 2022 were included. The clinical and histological characteristics of the AIH group were retrospectively analyzed and compared with those of the other four groups.
UNASSIGNED: A total of 208 children were included and divided into AIH group (18 patients), DILI group (38 patients), gene deficiency group (44 patients), infectious liver disease group (74 patients), and other etiology group (34 patients). The antinuclear antibodies (ANA) ≥ 1:320 rate was significantly higher in the AIH compared to the other four groups after multiple testing correction (p < 0.0125), while patients with positive antibodies to liver-kidney microsomal-1 (anti-LKM1, n = 3) and smooth muscle antibodies (SMA, n = 2) were only observed in the AIH group. The positive rates of antibodies to liver cytosol type1 (anti-LC1) and Ro52 were higher than those in the other four groups. The serum immunoglobulin G (IgG) and globulin levels, as well as the proportions of portal lymphoplasmacytic infiltration, lobular hepatitis with more than moderate interface hepatitis, and lobular hepatitis with lymphoplasmacytic infiltration, were significantly higher in the AIH group than in the other four groups after multiple testing correction (p < 0.0125). The cirrhosis rate in the AIH group was higher than that in the DILI and infectious liver disease groups (p < 0.0125). Both the simplified (AUC > 0.73) and the revised systems (AUC > 0.93) for AIH have good diagnostic performance, with the latter being superior (p < 0.05).
UNASSIGNED: Positive autoantibodies (ANA ≥ 1:320 or anti-LKM1 positive, or accompanied by SMA, anti-LC1 or Ro-52 positive) and elevated serum IgG or globulin levels contribute to early recognition of AIH. The presence of lobular hepatitis with more than moderate interface hepatitis and lymphoplasmacytic infiltration contribute to the diagnosis of AIH.
UNASSIGNED: All children with AIH-like liver disease at our center from January 2013 to December 2022 were included. The clinical and histological characteristics of the AIH group were retrospectively analyzed and compared with those of the other four groups.
UNASSIGNED: A total of 208 children were included and divided into AIH group (18 patients), DILI group (38 patients), gene deficiency group (44 patients), infectious liver disease group (74 patients), and other etiology group (34 patients). The antinuclear antibodies (ANA) ≥ 1:320 rate was significantly higher in the AIH compared to the other four groups after multiple testing correction (p < 0.0125), while patients with positive antibodies to liver-kidney microsomal-1 (anti-LKM1, n = 3) and smooth muscle antibodies (SMA, n = 2) were only observed in the AIH group. The positive rates of antibodies to liver cytosol type1 (anti-LC1) and Ro52 were higher than those in the other four groups. The serum immunoglobulin G (IgG) and globulin levels, as well as the proportions of portal lymphoplasmacytic infiltration, lobular hepatitis with more than moderate interface hepatitis, and lobular hepatitis with lymphoplasmacytic infiltration, were significantly higher in the AIH group than in the other four groups after multiple testing correction (p < 0.0125). The cirrhosis rate in the AIH group was higher than that in the DILI and infectious liver disease groups (p < 0.0125). Both the simplified (AUC > 0.73) and the revised systems (AUC > 0.93) for AIH have good diagnostic performance, with the latter being superior (p < 0.05).
UNASSIGNED: Positive autoantibodies (ANA ≥ 1:320 or anti-LKM1 positive, or accompanied by SMA, anti-LC1 or Ro-52 positive) and elevated serum IgG or globulin levels contribute to early recognition of AIH. The presence of lobular hepatitis with more than moderate interface hepatitis and lymphoplasmacytic infiltration contribute to the diagnosis of AIH.
摘要：
■患有自身免疫性肝炎（AIH）的儿童通常表现出与其他肝病相似的症状。本研究包括AIH与其他四种AIH样肝病的临床和组织学特征之间的比较[即，药物性肝损伤（DILI），基因缺陷,感染性肝病和其他肝病病因]，以及对AIH评分系统诊断性能的评估。
■包括2013年1月至2022年12月在我们中心的所有AIH样肝病儿童。回顾性分析AIH组的临床和组织学特征，并与其他四组进行比较。
■共有208名儿童被纳入AIH组（18名患者），DILI组（38例），基因缺陷组（44例），感染性肝病组（74例），和其他病因组（34例）。多重测试校正后，AIH的抗核抗体（ANA）≥1：320率明显高于其他四组（p<0.0125），而肝肾微粒体-1（抗LKM1，n=3）和平滑肌抗体（SMA，n=2）仅在AIH组中观察到。肝细胞溶胶1型（抗LC1）和Ro52抗体的阳性率高于其他四组。血清免疫球蛋白G（IgG）和球蛋白水平，以及门静脉淋巴浆细胞浸润的比例，小叶性肝炎伴中度以上界面性肝炎，和小叶性肝炎伴淋巴浆细胞浸润，在多重测试校正后，AIH组明显高于其他四组(p<0.0125)。AIH组肝硬化发生率高于DILI和感染性肝病组(p<0.0125)。AIH的简化系统（AUC>0.73）和修订系统（AUC>0.93）均具有良好的诊断性能，后者优于后者(p<0.05)。
■自身抗体阳性（ANA≥1：320或抗LKM1阳性，或伴有SMA，抗LC1或Ro-52阳性）和血清IgG或球蛋白水平升高有助于AIH的早期识别。小叶性肝炎伴中度以上界面性肝炎和淋巴浆细胞浸润有助于AIH的诊断。
■包括2013年1月至2022年12月在我们中心的所有AIH样肝病儿童。回顾性分析AIH组的临床和组织学特征，并与其他四组进行比较。
■共有208名儿童被纳入AIH组（18名患者），DILI组（38例），基因缺陷组（44例），感染性肝病组（74例），和其他病因组（34例）。多重测试校正后，AIH的抗核抗体（ANA）≥1：320率明显高于其他四组（p<0.0125），而肝肾微粒体-1（抗LKM1，n=3）和平滑肌抗体（SMA，n=2）仅在AIH组中观察到。肝细胞溶胶1型（抗LC1）和Ro52抗体的阳性率高于其他四组。血清免疫球蛋白G（IgG）和球蛋白水平，以及门静脉淋巴浆细胞浸润的比例，小叶性肝炎伴中度以上界面性肝炎，和小叶性肝炎伴淋巴浆细胞浸润，在多重测试校正后，AIH组明显高于其他四组(p<0.0125)。AIH组肝硬化发生率高于DILI和感染性肝病组(p<0.0125)。AIH的简化系统（AUC>0.73）和修订系统（AUC>0.93）均具有良好的诊断性能，后者优于后者(p<0.05)。
■自身抗体阳性（ANA≥1：320或抗LKM1阳性，或伴有SMA，抗LC1或Ro-52阳性）和血清IgG或球蛋白水平升高有助于AIH的早期识别。小叶性肝炎伴中度以上界面性肝炎和淋巴浆细胞浸润有助于AIH的诊断。
