Wasserman Richard L
Allergy Partners of North Texas, Medical City Children's Hospital, Dallas, TX, USA.
Immunotherapy. 2017 Sep;9(12):1035-1050. doi: 10.2217/imt-2017-0092. Epub 2017 Sep 5.
Most primary immunodeficiency diseases (PIDDs) resulting in antibody deficiency require intravenous or subcutaneous immunoglobulin G (SCIG) replacement therapy. The flow and distribution of SCIG to the vasculature is impeded by the glycosaminoglycan hyaluronan in the extracellular matrix, which limits the infusion rate and volume per site, necessitating frequent infusions and multiple infusion sites. Hyaluronidase depolymerizes hyaluronan and is a spreading factor for injectable biologics. Recombinant human hyaluronidase (rHuPH20) increases SCIG absorption and dispersion. In patients with PIDD, SCIG facilitated with rHuPH20 (IGHy) has been shown to prevent infections, be well-tolerated and reduce infusion frequency and number of infusion sites as compared with conventional SCIG. This article reviews IGHy clinical studies and real-world practice data in patients with PIDD.
大多数导致抗体缺陷的原发性免疫缺陷病（PIDD）需要静脉注射或皮下注射免疫球蛋白G（SCIG）替代疗法。细胞外基质中的糖胺聚糖透明质酸会阻碍SCIG向脉管系统的流动和分布，这限制了每个部位的输注速率和体积，因此需要频繁输注且使用多个输注部位。透明质酸酶可使透明质酸解聚，是可注射生物制品的扩散因子。重组人透明质酸酶（rHuPH20）可增加SCIG的吸收和分散。在PIDD患者中，与传统SCIG相比，使用rHuPH20辅助的SCIG（IGHy）已被证明可预防感染、耐受性良好，并可减少输注频率和输注部位数量。本文综述了PIDD患者中IGHy的临床研究和真实世界实践数据。
