Division of Rheumatology, Allergy and Immunology, UC San Diego, School of Medicine, La Jolla, CA, USA.
Best Pract Res Clin Rheumatol. 2012 Aug;26(4):425-38. doi: 10.1016/j.berh.2012.07.001.
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fascinating condition recognised as a systemic disease in 2003 [1,2]. The first link between autoimmunity affecting the pancreas, elevated serum IgG4 concentrations and large numbers of IgG4-positive plasma cells in pancreatic tissue was described only 2 years earlier [3]. Since then, many diseases that have long been viewed organ-specific are now considered within the spectrum of IgG4-RD. Practically any organ can be affected, having in common a key pathological feature consisting in dense lymphocyte and plasma cell infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and often an elevated serum IgG4 concentration. While good clinical response to steroid therapy is observed, immunosuppressive or B-cell depleting therapy can be required. It is important to distinguish the IgG4-RD from traditional organ-specific autoimmune disease to guide therapy.
免疫球蛋白 G4（IgG4）相关疾病（IgG4-RD）是一种引人入胜的疾病，于 2003 年被确认为一种系统性疾病[1,2]。自身免疫影响胰腺、血清 IgG4 浓度升高和胰腺组织中大量 IgG4 阳性浆细胞之间的最初联系仅在 2 年前描述[3]。自那时以来，许多长期被视为器官特异性的疾病现在被认为属于 IgG4-RD 谱。几乎任何器官都可能受到影响，其共同的关键病理特征包括密集的淋巴细胞和浆细胞浸润，富含 IgG4 阳性浆细胞、席纹状纤维化，并且常常伴有血清 IgG4 浓度升高。虽然观察到对类固醇治疗的良好临床反应，但可能需要免疫抑制或 B 细胞耗竭治疗。区分 IgG4-RD 与传统的器官特异性自身免疫性疾病对于指导治疗很重要。
