Department of Gastroenterology, Hepatology, Feeding Disorders and Paediatrics, The Children's Memorial Health Institute, Warsaw, Poland.
Department of Microbiology and Clinical Immunology, The Children's Memorial Health Institute, Warsaw, Poland.
Pol J Pathol. 2024;75(2):153-156. doi: 10.5114/pjp.2024.141231.
The IgG4-associated autoimmune hepatitis (IgG4-AIH) is a newly proposed disease entity characterised by the accumulation of the IgG4-expressing plasma cells in the liver. Its pathophysiology and clinical significance remain unclear and have poor evidence in the paediatric population. Thus, our study aims at comparing the group of paediatric patients with classical AIH and the IgG4-AIH. We carried out a retrospective analysis of 23 children (median age 8.5 years) diagnosed with AIH, who were compared according to the presence of IgG4-positive plasma cells in the liver biopsy. IgG4-AIH was defined if 10 or more IgG4 positive plasma cells/high-power field were found in the biopsy. The presence of the IgG4 component seems to be clinically insignificant. That is why, the conventional immunosuppressive protocol should be considered the standard treatment in the case of the IgG4-associated AIH.
IgG4 相关性自身免疫性肝炎（IgG4-AIH）是一种新提出的疾病实体，其特征在于 IgG4 表达浆细胞在肝脏中的积累。其病理生理学和临床意义尚不清楚，在儿科人群中证据不足。因此，我们的研究旨在比较儿童经典 AIH 组和 IgG4-AIH 组。我们对 23 名被诊断为 AIH 的儿童（中位年龄 8.5 岁）进行了回顾性分析，根据肝活检中是否存在 IgG4 阳性浆细胞进行比较。如果在活检中发现 10 个或更多 IgG4 阳性浆细胞/高倍视野，则定义为 IgG4-AIH。IgG4 成分的存在似乎在临床上并不重要。因此，在 IgG4 相关性 AIH 的情况下，应考虑常规免疫抑制方案作为标准治疗。
