Humoral immunodeficiency diseases represent a heterogeneous group of disorders that require long-term therapies. Thus, the treatment provided must not only be effective but also safe and well tolerated. In this paper, we report our data on the efficacy, safety, and tolerability of recombinant human hyaluronidase-facilitated subcutaneous infusion of immunoglobulin (Ig) (fSCIG; HyQvia(^®)) in immunodeficiency patients. We collected retrospective data from 30 patients with primary and secondary immunodeficiency diseases in therapy with fSCIG from September 2014 to December 2019. We evaluated the efficacy of the therapy, taking into account serum IgG values during follow-up and the number of annual infectious events and serious bacterial infections reported by patients. Safety was assessed on the basis of the number and intensity of adverse events (AEs) and local reactions reported. Our real-life data suggest that long-term repeated self-administration of recombinant human hyaluronidase-facilitated subcutaneous infusion of immunoglobulins results in a reduced rate of infectious events if compared to the pre-treatment rate. Both AEs and local reactions are mild to moderate and were never reasons for treatment discontinuation. Therapy with HyQvia shows prolonged efficacy and good tolerability; these aspects, together with the possibility of self-administration at home, minimize the impact the illness has on patients.

中文翻译：

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在免疫缺陷疾病中重组人透明质酸酶促进的免疫球蛋白（Ig）皮下输注（fSCIG; HyQvia（®））的长期疗效，安全性和耐受性：单中心经验的真实数据。

体液免疫缺陷疾病代表需要长期治疗的一组异类疾病。因此，所提供的治疗不仅必须有效，而且必须安全且耐受良好。在本文中，我们报告了有关重组人透明质酸酶促进皮下注射免疫球蛋白（Ig）（fSCIG; HyQvia（^®）的功效，安全性和耐受性的数据））在免疫缺陷患者中。我们收集了2014年9月至2019年12月接受fSCIG治疗的30例原发性和继发性免疫缺陷疾病患者的回顾性数据。我们评估了该治疗的有效性，并考虑了随访期间的血清IgG值以及年度感染事件的数量和患者报告严重细菌感染。根据不良事件（AE）的数量和强度以及报告的局部反应评估安全性。我们的现实生活数据表明，与治疗前相比，重组人透明质酸酶促进的皮下免疫球蛋白输注长期重复自我给药可降低感染事件的发生率。不良事件和局部反应均为轻度至中度，并且从来都不是中断治疗的原因。HyQvia疗法显示出延长的疗效和良好的耐受性；这些方面，加上在家自我管理的可能性，可将疾病对患者的影响降至最低。