Immunoglobulin G4-related disease (IgG4-RD) is a newly acknowledged entity, characterized by an immune-mediated fibro-inflammatory process affecting virtually all organs, with infiltration of IgG4⁺ bearing plasma cells. Until today the pathogenesis of IgG4-RD remains unknown. Treatment with anti-CD20 monoclonal antibodies efficiently induced remission and attenuated the secretory phenotype of myofibroblasts responsible of uncontrolled collagen deposition. This supports the pathogenic role of the adaptive immunity, particularly B cell compartment and B cell/T cell interaction. Latest studies have also highlighted the importance of innate immune system that has been underestimated before and the key role of a specific T cell subset, T follicular helper cells that are involved in IgG4-class-switching and plasmablast differentiation. In this review, we aim to review the most recent knowledge of innate immunity, T and B cells involvement in IgG4-RD, and introduce tertiary lymphoid organs (TLO) as a potential marker of relapse in this condition.

免疫球蛋白G4相关疾病（IgG4-RD）是新近公认的实体，其特征是免疫介导的纤维炎性过程影响了几乎所有器官，并伴有IgG4 ^+的浸润。携带浆细胞。直到今天，IgG4-RD的发病机理仍然未知。用抗CD20单克隆抗体治疗可有效诱导缓解，并减弱负责不受控制的胶原蛋白沉积的成肌纤维细胞的分泌表型。这支持了适应性免疫的致病作用，尤其是B细胞区室和B细胞/ T细胞相互作用。最新研究还强调了之前被低估的先天免疫系统的重要性，以及特定T细胞亚群（参与IgG4类转换和成浆细胞分化的T滤泡辅助细胞）的关键作用。在这篇综述中，我们旨在综述关于先天免疫，T细胞和B细胞参与IgG4-RD的最新知识，并引入第三类淋巴器官（TLO）作为这种情况下复发的潜在标志。