Service de Néphrologie et Dialyses, AP-HP, Hôpital Tenon, Paris, France.
Nephrol Dial Transplant. 2011 Jun;26(6):2047-50. doi: 10.1093/ndt/gfr097. Epub 2011 Mar 15.
IgG4-related systemic disease is a protean disorder that covers a wide variety of lesions. We report on a patient with tubulointerstitial nephritis, lymphadenopathies, sialadenitis and retroperitoneal fibrosis. The salivary gland and kidney interstitium were infiltrated with B lymphocytes and T lymphocytes and IgG3(+) and IgG4(+) plasma cells. The overexpression of IgG1 and IgG3, in addition to IgG4, the unusual abundance of interfollicular plasma cells and CD4(+) T cells in germinal centres of lymph nodes, and the dramatic response to rituximab point to possible roles of follicular helper T cells in enhancing a skewed B-cell terminal maturation and of CD20(+) B cells in disease progression.
IgG4 相关系统性疾病是一种多系统疾病，可引起多种病变。我们报告一例 tubulointerstitial nephritis、淋巴结病、唾液腺炎和腹膜后纤维化患者。患者的唾液腺和肾脏间质有 B 淋巴细胞、T 淋巴细胞和 IgG3(+)、IgG4(+)浆细胞浸润。IgG1 和 IgG3 的过度表达，除 IgG4 外，淋巴结生发中心滤泡间浆细胞和 CD4(+)T 细胞异常丰富，以及利妥昔单抗治疗的显著疗效，提示滤泡辅助 T 细胞可能在增强 B 细胞终末成熟的偏倚和 CD20(+)B 细胞在疾病进展中的作用。
