Jordan S C
Department of Pediatrics, Cedars-Sinai Medical Center, Los Angeles, California 90048.
Clin Immunol Immunopathol. 1989 Nov;53(2 Pt 2):S164-9. doi: 10.1016/0090-1229(89)90082-2.
Immune complexes (ICs) are felt to be of primary pathological importance in the mediation of many human glomerular diseases. This is based on the demonstration of Ig and C' in renal cortex tissue of affected individuals. Systemic lupus erythematosus (SLE) is a prototype IC disease where ICs have been demonstrated in target tissues. Moreover, glomerulonephritis (GN) is a common feature of many autoimmune and infectious diseases associated with IC generation. Current therapeutic alternatives are restricted to immunosuppressive agents. Tomino et al. (Clin. Exp. Immunol. 58, 42, 1984) demonstrated that glomerular IC deposits could be solubilized with HGG. Palla et al. (Clin. Nephrol. 26, 314, 1986) treated four membranous nephritis patients with IVGG and had dramatic resolution of proteinuria in three of them. Gaedlicke et al. (Blut 48, 387, 1984) reported improvement in vasculitis in one of two patients treated with IVGG. We have experienced exacerbation of GN with IVGG therapy in two SLE patients. IVGG is useful in treating the common variable immunodeficiency that occurs in some SLE patients and in treating the immunodeficiency associated with florid nephrotic syndrome. IVGG given to one patient with Henoch-Schönlein purpura resulted in the onset of gross hematuria. In sum, IVGG may be useful in treating specific IC renal diseases by solubilization of circulating or in situ ICs but definitive proof is lacking. In other situations, IVGG may exacerbate the glomerulonephritis, possibly through enhanced IC formation. Furthermore, IVGG may induce modulation of immune responses by induction of auto-anti-idiotypic immunity.
免疫复合物（ICs）被认为在许多人类肾小球疾病的介导中具有主要的病理重要性。这是基于在受影响个体的肾皮质组织中证实存在免疫球蛋白（Ig）和补体（C'）。系统性红斑狼疮（SLE）是一种典型的IC疾病，已在靶组织中证实存在ICs。此外，肾小球肾炎（GN）是许多与IC生成相关的自身免疫性疾病和感染性疾病的常见特征。目前的治疗选择仅限于免疫抑制剂。富美野等人（《临床与实验免疫学》第58卷，第42页，1984年）证明肾小球IC沉积物可用人γ球蛋白（HGG）溶解。帕拉等人（《临床肾脏病学》第26卷，第314页，1986年）用静脉注射人γ球蛋白（IVGG）治疗了4例膜性肾病患者，其中3例蛋白尿得到显著缓解。盖德利克等人（《血液》第48卷，第387页，1984年）报告，在2例接受IVGG治疗的患者中，有1例血管炎病情改善。我们有2例SLE患者在接受IVGG治疗后GN病情加重。IVGG可用于治疗一些SLE患者出现的常见可变免疫缺陷以及与严重肾病综合征相关的免疫缺陷。给1例过敏性紫癜患者注射IVGG后出现肉眼血尿。总之，IVGG可能通过溶解循环中的或原位的ICs来治疗特定的IC肾病，但缺乏确凿证据。在其他情况下，IVGG可能会加重肾小球肾炎，可能是通过增强IC的形成。此外，IVGG可能通过诱导自身抗独特型免疫来诱导免疫反应的调节。
