Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
Department of Nephrology and Urology, Fukui Red Cross Hospital, Fukui, Fukui, Japan.
Expert Rev Clin Immunol. 2024 Jan-Jun;20(1):97-119. doi: 10.1080/1744666X.2023.2274358. Epub 2024 Jan 8.
IgG4-related disease (IgG4-RD) is an immune-mediated systemic fibroinflammatory condition characterized by serum IgG4 elevation and IgG4-positive plasma cell infiltration into various organs. It generally occurs in elderly males. Pediatric cases have been reported, albeit rarely, accordingly lack of recognition of such cases could delay therapeutic intervention leading to poorer outcomes.
The present review is a descriptive review of all published case reports, cohort studies, and reviews of pediatric IgG4-RD listed in PubMed. Characteristics of pediatric IgG4-RD were clarified, including sex, organ involvement, serological and histological findings, and treatment. We assessed how many published cases met current classification and comprehensive diagnostic criteria.
The characteristics of pediatricIgG4-RD differed from adult IgG4-RD in terms of sex and involved organs. There was no clear male dominance in numbers of cases, and surface organ involvement such as ophthalmic diseases were more common in the pediatric IgG4-RD. Organ involvement tended to be indolent and unilateral, causing difficulty in definitively diagnosing pediatric IgG4-RD. Only about 20% of published cases met IgG4-RD classification or comprehensive diagnostic criteria. Physicians should be careful in diagnosing pediatric IgG4-RD after excluding mimickers. International collaboration toward high-quality evidence to support diagnosis and treatment of pediatric IgG4-RD is advised.
IgG4 相关疾病（IgG4-RD）是一种免疫介导的系统性纤维炎症性疾病，其特征是血清 IgG4 升高和 IgG4 阳性浆细胞浸润到各种器官。它通常发生在老年男性中。尽管儿科病例有报道，但很少见，因此，如果对这些病例认识不足，可能会延迟治疗干预，导致预后较差。
本综述是对 PubMed 上列出的所有已发表的儿科 IgG4-RD 病例报告、队列研究和综述的描述性综述。明确了儿科 IgG4-RD 的特征，包括性别、器官受累、血清学和组织学发现以及治疗。我们评估了有多少已发表的病例符合当前的分类和综合诊断标准。
儿科 IgG4-RD 的特征在性别和受累器官方面与成人 IgG4-RD 不同。在病例数量上，没有明显的男性优势，而儿科 IgG4-RD 中更常见眼部等表面器官受累。器官受累往往是惰性和单侧的，这使得明确诊断儿科 IgG4-RD 变得困难。只有约 20%的已发表病例符合 IgG4-RD 分类或综合诊断标准。在排除类似疾病后，医生应谨慎诊断儿科 IgG4-RD。建议国际合作，以获得支持儿科 IgG4-RD 诊断和治疗的高质量证据。
