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IgG4相关性疾病(IgG4-RD)是一种纤维炎症性疾病,可累及多器官、多系统,其疾病谱逐渐扩大。目前虽针对其发病机制的研究众多,但仍尚未明确。该病临床谱复杂,虽目前已有多套诊断标准,但仍有诸多局限性,缺乏理想的生物标志物(尤其是外周血生物标志物)是一大困惑。浆母细胞是一类介于活化B细胞与成熟浆细胞之间的B细胞亚群,活动性IgG4-RD患者外周血浆母细胞水平明显升高,较血清IgG4水平更适于作为疾病标志物。IgG4-RD中,浆母细胞的质和量与其他自身免疫性疾病存在差异。
IgG4-related disease (IgG4-RD) is a fibrotic inflammatory disease that can affect multiple organs, multiple systems, and its disease spectrum is gradually expanding. Although the current study of its pathogenesis of many, but still not yet clear. The clinical spectrum of the disease is complex. Although there are already a number of diagnostic criteria, there are still many limitations. The lack of ideal biomarkers (especially peripheral blood biomarkers) is a big confusion. Plasmacytoma is a subset of B-cell subsets between activated B cells and mature plasma cells. Peripheral plasmacytoplasm activity is significantly elevated in patients with active IgG4-RD and is more suitable as a disease marker than serum IgG4 levels. The quality and quantity of plasmablasts differs from other autoimmune diseases in IgG4-RD.