Abstract：
IgG4-related disease (IgG4-RD) is a systemic and chronic inflammatory disorder that can affect every part of the body. The formation of tertiary lymphoid tissues (TLT) in the affected organs may be a key phenomenon in understanding the pathogenesis of this disease because T follicular helper (Tfh) 2 cells play an important role in IgG4 class switching within TLT in the affected organs or tissues. TLT formation leads to the formation of masses or swelling of the affected organs. Interleukin (IL)-4 and IL-10 are critical cytokines for IgG4-class switching and are produced in TLT. Other factors, such as CD4-positive (CD4+) cytotoxic T cells, M2 macrophages, and LAG3+ Tfh cells, have been identified as disease-specific contributors to lesion formation. In this review, I describe the current knowledge necessary to understand the pathogenesis of this disease and recent developments in treatment strategies beyond B-cell depletion therapy.
摘要：
IgG4相关疾病（IgG4-RD）是一种全身性和慢性炎症性疾病，可影响身体的每个部位。受累器官中三级淋巴组织（TLT）的形成可能是了解该疾病发病机理的关键现象，因为滤泡辅助性T（Tfh）2细胞在受累器官或组织中的TLT内的IgG4类别转换中起重要作用。TLT形成导致受累器官的肿块或肿胀的形成。白细胞介素（IL）-4和IL-10是IgG4类别转换的关键细胞因子，并在TLT中产生。其他因素,如CD4阳性（CD4+）细胞毒性T细胞，M2巨噬细胞，和LAG3+Tfh细胞，已被确定为病变形成的疾病特异性贡献者。在这次审查中,我描述了了解这种疾病的发病机理所必需的当前知识，以及B细胞耗竭疗法以外的治疗策略的最新发展。
