Steven and Alexandra Cohen Children's Medical Center of New York, North Shore-Long Island Jewish Health System, 865 Northern Blvd, Suite 101, Great Neck, NY 11021, USA.
J Clin Immunol. 2013 Jan;33 Suppl 2:S90-4. doi: 10.1007/s10875-012-9838-1. Epub 2012 Dec 28.
The treatment of primary immunodeficiency disease (PIDD) patients with immunoglobulin obtained from healthy controls, given intravenously, is a relatively recent event, having first been given in 1981. Intravenous immunoglobulin (IVIG) replacement in PIDD has been shown to prevent serious/recurrent infections because higher IgG levels can be obtained through IV administration, as opposed to the intramuscular route. Significant variation in IgG levels in controls is dependent on age and sex, which provides the rationale for the concept that there is a "biological IgG trough/level", hereafter called biological IgG level, in PIDD, as there is in healthy controls. Each PIDD patient has a biological IgG level that can be altered by comorbid conditions that evoke IgG loss or changes in metabolism/catabolism. The pharmacokinetic comparison of IVIG vs. SCIG demonstrates the various benefits of each in treating PIDD. Acutely ill PIDD patients should only receive IVIG. "Rush" SCIG treatment can also be used to attain the biological IgG level, but for less emergent care of PIDD. Finally, future opportunities exist to enhance IgG replacement in PIDD, including microbe-specific IgG and IgG subclass-specific enriched preparations.
原发性免疫缺陷病(PIDD)患者使用从健康对照者获得的免疫球蛋白进行静脉注射治疗是相对较新的方法,首次应用于 1981 年。静脉注射免疫球蛋白(IVIG)替代治疗 PIDD 已被证明可预防严重/复发性感染,因为通过静脉途径给药可以获得更高的 IgG 水平,而不是通过肌肉途径。对照者中 IgG 水平的显著差异取决于年龄和性别,这为 PIDD 存在“生物学 IgG 谷值/水平”的概念提供了依据,就像健康对照者一样。每个 PIDD 患者都有一个生物学 IgG 水平,可通过引起 IgG 丢失或代谢/分解代谢变化的合并症改变。IVIG 与 SCIG 的药代动力学比较表明了每种治疗 PIDD 的优势。急性疾病 PIDD 患者只能接受 IVIG。“冲击”SCIG 治疗也可用于达到生物学 IgG 水平,但用于 PIDD 的紧急护理较少。最后,存在增强 PIDD 中 IgG 替代的未来机会,包括针对特定微生物的 IgG 和 IgG 亚类特异性富集制剂。