Improved Quality of Life, Immunoglobulin G Levels, and Infection Rates in Patients with Primary Immunodeficiency Diseases during Self-Treatment with Subcutaneous Immunoglobulin G
Improved Quality of Life, Immunoglobulin G Levels, and Infection Rates in Patients with Primary Immunodeficiency Diseases during Self-Treatment with Subcutaneous Immunoglobulin G
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DOI：
10.1097/smj.0b013e3181eba6ea
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发表时间：
2010-09-01
影响因子：
1.1
通讯作者：
Bergman, Garrett E.
中科院分区：
文献类型：
--
作者：
Berger, Melvin;Murphy, Elyse;Bergman, Garrett E.
目的：原发免疫缺陷病(PIDD)包括一大类遗传异质性疾病，使患者容易发生严重和慢性/复发感染，并降低生活质量(QOL)。静脉免疫球蛋白(IVIG)治疗改善了PIDD患者的健康状况；然而，静脉途径的需要和潜在的严重副作用经常需要在医疗机构中使用。我们评估了自体皮下注射免疫球蛋白(SCIG)对PIDD患者健康相关生活质量、严重细菌感染率和所有其他感染的长期(12个月)体验。方法：对42例PIDD患者和9例接受临床IVIG治疗的PIDD患者进行在家自我应用SCIG的培训。每隔一段时间记录患者的生活质量(SF-36(R)和CHQ-PF50问卷)、严重细菌感染、血清免疫球蛋白G水平、总感染情况和不良事件发生率。结果：所有患者的总体健康状况均有改善(成人P=0.047，儿童P=0.037)。成年人在身体疼痛和活力评估方面也有明显改善，父母对个人和家庭活动的看法也有所改善。血清免疫球蛋白水平维持在比静脉注射免疫球蛋白之前的谷值高25%的平均水平。总共有162例感染，年感染率为3.42例/例，但仅观察到1例严重细菌感染(0.03例/例/年)。患者平均每年缺勤4.5天。结论：家庭SCIG治疗是安全的，可改善患者对一般健康的认知，提高血清免疫球蛋白水平，降低感染率和缺课天数。
Objectives: Primary immunodeficiency diseases (PIDDs) include a large class of genetically heterogeneous disorders which predispose patients to significant risk of serious and chronic/recurrent infections, as well as reduced quality of life (QoL). Intravenous immunoglobulin (IVIG) therapy improves the well being of PIDD patients; however, the need for venous access and potentially severe side effects frequently require administration in medical facilities. We evaluated the long-term (12-month) experience with home-based self infusions of subcutaneous immune globulin (SCIG) in patients with PIDD on health-related QoL, rates of serious bacterial infections, and all other infections.Methods: Adults (n = 42) and children (n = 9) with PIDD, previously treated with clinic-based IVIG, were trained to self administer SCIG at home. QoL (SF-36 (R) and CHQ-PF50 questionnaires), serious bacterial infections, serum immunoglobulin G (IgG) levels, overall infections, and incidence of adverse events were recorded at predetermined intervals.Results: All patients had improved perceptions of general health (adults P = 0.047, children P = 0.037). Adults also had marked improvement in the bodily pain and vitality assessments, and parents had improved perceptions of personal and family activities. Serum IgG levels were maintained at mean levels 25% higher than previous troughs on IVIG. There were 162 infections overall for an annual rate of 3.42/patient, but only 1 serious bacterial infection was observed (0.03/patient/yr). An average of 4.5 days/yr was missed from work or school per patient.Conclusions: Home SCIG therapy was safe and led to improved perceptions of general health, higher serum IgG levels, and very low rates of infections and days missed from work/school.