Department of Clinical Immunology, Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom.
J Allergy Clin Immunol. 2010 Jun;125(6):1354-1360.e4. doi: 10.1016/j.jaci.2010.02.040. Epub 2010 May 14.
Common variable immunodeficiency disorders (CVIDs) are the most common forms of symptomatic primary antibody failure in adults and children. Replacement immunoglobulin is the standard treatment, although there are few consistent data on optimal dosages and target trough IgG levels required for infection prevention.
To provide data to support the hypothesis that each patient requires an individual dose of therapeutic immunoglobulin to prevent breakthrough infections and that efficacious trough IgG levels vary between patients.
Data, collected prospectively from a cohort of 90 patients with confirmed CVIDs from 1 center over a follow-up period of 22 years, was validated and analyzed. Immunoglobulin doses had been adjusted in accordance with infections rather than to achieve a particular trough IgG level. Doses to achieve infection-free periods were determined and resultant trough levels analyzed. A smaller group of patients with X-linked agammaglobulinemia was analyzed for comparison.
Patients with a CVID had a range of trough IgG levels that prevented breakthrough bacterial infections (5-17 g/L); viral and fungal infections were rare. Doses of replacement immunoglobulin to prevent breakthrough infections ranged from 0.2 to 1.2 g/kg/mo. Those with proven bronchiectasis or particular clinical phenotypes required higher replacement doses. Patients with X-linked agammaglobulinemia showed a similar range of IgG levels to stay infection-free (8-13 g/L).
These data offer guidance regarding optimal doses and target trough IgG levels in individual patients with CVIDs with or without bronchiectasis and for particular clinical phenotypes. The goal of replacement therapy should be to improve clinical outcome and not to reach a particular IgG trough level.
普通变异性免疫缺陷病（CVID）是成人和儿童中最常见的症状性原发性抗体功能障碍。替代免疫球蛋白是标准治疗方法，尽管关于预防感染所需的最佳剂量和目标 IgG 谷值水平的数据很少。
提供数据支持以下假设：每位患者都需要个体化的治疗性免疫球蛋白剂量来预防突破性感染，并且有效的 IgG 谷值水平在患者之间存在差异。
从一个中心的 90 例确诊 CVID 患者的队列中前瞻性收集数据，该队列在 22 年的随访期间进行了验证和分析。免疫球蛋白剂量是根据感染进行调整的，而不是为了达到特定的 IgG 谷值水平。确定了实现无感染期的剂量，并分析了相应的 IgG 谷值。对一小部分 X 连锁无丙种球蛋白血症患者进行了分析比较。
CVID 患者的 IgG 谷值范围差异较大，可以预防细菌性突破性感染（5-17 g/L）；病毒和真菌感染很少见。预防突破性感染所需的替代免疫球蛋白剂量范围为 0.2-1.2 g/kg/mo。有支气管扩张症或特定临床表型的患者需要更高的替代剂量。X 连锁无丙种球蛋白血症患者保持无感染所需的 IgG 水平相似（8-13 g/L）。
这些数据为有或无支气管扩张症以及特定临床表型的 CVID 患者的最佳剂量和目标 IgG 谷值水平提供了指导。替代治疗的目标应该是改善临床结果，而不是达到特定的 IgG 谷值水平。
