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Effectiveness of low-dose intravenous immunoglobulin therapy in minor primary antibody deficiencies: A 2-year real-life experience
Clinical & Experimental Immunology ( IF 4.6 ) Pub Date : 2021-06-01 , DOI: 10.1111/cei.13629
Emanuele Vivarelli 1 , Andrea Matucci 1 , Susanna Bormioli 1 , Paola Parronchi 2 , Francesco Liotta 2 , Lorenzo Cosmi 2 , Fabio Almerigogna 1 , Alessandra Vultaggio 1
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Primary antibody deficiencies (PAD) are the most prevalent group of primary immunodeficiencies (PID) in adults and immunoglobulin replacement therapy (IRT) is the mainstay therapy to improve clinical outcomes. IRT is, however, expensive and, in minor PAD, clear recommendations concerning IRT are lacking. We conducted a retrospective real-life study to assess the effectiveness of low-dose IRT in minor PAD on 143 patients fulfilling European Society for Immunodeficiencies (ESID) diagnostic criteria for immunoglobulin (Ig)G subclass deficiency (IgGSD) or unclassified antibody deficiency (UAD). All patients were treated with intravenous low-dose IRT (0.14 ± 0.06 g/kg/month). Immunoglobulin (Ig) classes and IgG subclasses were measured at baseline and after 1 year of IRT. The annual rate of total infections, upper respiratory tract infections (URTI), lower respiratory tract infections (LRTI) and hospitalizations was measured at baseline and after 1 and 2 years of IRT. After 1 year of IRT significant improvement was demonstrated in: (a) serum IgG (787.9 ± 229.3 versus 929.1 ± 206.7 mg/dl; p < 0.0001); (b) serum IgG subclasses (IgG1 = 351.4 ± 109.9 versus 464.3 ± 124.1, p < 0.0001; IgG2 = 259.1 ± 140 versus 330.6 ± 124.9, p < 0.0001; IgG3 = 50.2 ± 26.7 versus 55.6 ± 28.9 mg/dl, p < 0.002); (c) annual rate of total infections (5.75 ± 3.87 versus 2.13 ± 1.74, p < 0.0001), URTI (1.48 ± 3.15 versus 0.69 ± 1.27; p < 0.005), LRTI (3.89 ± 3.52 versus 1.29 ± 1.37; < 0.0001) and hospitalizations (0.37 ± 0.77 versus 0.15 ± 0.5; p < 0.0002). The improvement persisted after 2 years of IRT. No significant improvement in URTI annual rate was noted in UAD and in patients with bronchiectasis. In conclusion, low-dose IRT can improve clinical outcomes in UAD and IgGSD patients, providing a potential economical advantage over the standard IRT dose.

中文翻译:

低剂量静脉注射免疫球蛋白治疗轻微原发性抗体缺陷的有效性:2 年真实生活经验

原发性抗体缺陷 (PAD) 是成人中最常见的原发性免疫缺陷 (PID) 组,免疫球蛋白替代疗法 (IRT) 是改善临床结果的主要疗法。然而,IRT 很昂贵,而且在轻微的 PAD 中,缺乏关于 IRT 的明确建议。我们进行了一项回顾性现实生活研究,以评估低剂量 IRT 对 143 名符合欧洲免疫缺陷协会 (ESID) 免疫球蛋白 (Ig)G 亚类缺陷 (IgGSD) 或未分类抗体缺陷 (UAD) 诊断标准的患者在轻微 PAD 中的有效性)。所有患者均接受静脉低剂量 IRT(0.14 ± 0.06 g/kg/月)治疗。在基线和 IRT 1 年后测量免疫球蛋白 (Ig) 类别和 IgG 亚类。年总感染率、上呼吸道感染 (URTI)、在基线和 IRT 1 年和 2 年后测量下呼吸道感染 (LRTI) 和住院率。1 年后 IRT 显着改善表现在: (a) 血清 IgG (787.9 ± 229.3929.1 ± 206.7 毫克/分升相比;p  < 0.0001); (b) 血清 IgG 亚类(IgG1 = 351.4 ± 109.9 vs 464.3 ± 124.1,p  < 0.0001;IgG2 = 259.1 ± 140 vs 330.6 ± 124.9,p  < 0.0001;IgG3 = 50.2 ± 26.7 vs 55.6 ± 28.9 mg/dl,p  < 0.002); (c) 年总感染率 (5.75 ± 3.87 vs 2.13 ± 1.74, p  < 0.0001), URTI (1.48 ± 3.15 vs 0.69 ± 1.27; p  < 0.005), LRTI (3.89 ± 3.52 vs 1.29 ± 1.37; < 0.0001 ) 和住院(0.37 ± 0.770.15 ± 0.5;p  < 0.0002)。在 IRT 2 年后,这种改善仍然存在。UAD 和支气管扩张患者的 URTI 年发病率没有显着改善。总之,低剂量 IRT 可以改善 UAD 和 IgGSD 患者的临床结果,与标准 IRT 剂量相比具有潜在的经济优势。
更新日期:2021-06-01
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