Prescribing Immunoglobulin Replacement Therapy for Patients with Non-classical and Secondary Antibody Deficiency: an Analysis of the Practice of Clinical Immunologists in the UK and Republic of Ireland
Prescribing Immunoglobulin Replacement Therapy for Patients with Non-classical and Secondary Antibody Deficiency: an Analysis of the Practice of Clinical Immunologists in the UK and Republic of Ireland
复制标题
DOI：
10.1007/s10875-017-0469-4
复制
发表时间：
2018-02-01
影响因子：
9.1
通讯作者：
Sorensen, Ricardo U.
中科院分区：
文献类型：
--
作者：
Edgar, John David M.;Richter, Alex G.;Sorensen, Ricardo U.
免疫学家越来越多地被要求评估非经典和二次抗体缺乏症患者，以确定他们对免疫球蛋白替代疗法（IGRT）的潜在需求。免疫球蛋白是一种有限的、昂贵的资源，对于这一广泛的患者群体没有明确的指导。这项调查的目的是建立什么因素影响的决定开始IGRT在成人患者，当诊断标准的一级抗体缺乏症是不fulfiled. Under的主持下，英国原发性免疫缺陷网络（UKPIN），成立了一个研究小组，分发了一份在线问卷咨询机构在英国和爱尔兰。结果提供了一个快照的71%的顾问免疫学家，从30 centers.In的重要性，影响决定开始IGRT的因素包括感染，血清IgG水平，支气管扩张，放射学证实的肺炎，阳性痰培养的数量，抗生素疗程数，和免疫研究的结果入院人数。最常用的试验疫苗是Pneumovax 2/3，在4周时测量了血清型特异性应答，在2/3的血清型中测量的阈值为0.35 μ g/ml。86%的患者在考虑IGRT之前接受了预防性抗生素试验。IGRT试验的有效性在6至12个月之间进行评估。使用临床病史、感染证据和疫苗接种试验进行诊断的组合，在临床实践中具有一致性。然而，在实施这一做法方面存在一些差异，特别是在疫苗选择和疫苗接种反应评估方面。
Immunologists are increasingly being asked to assess patients with non-classical and secondary antibody deficiency to determine their potential need for immunoglobulin replacement therapy (IGRT). Immunoglobulin is a limited, expensive resource and no clear guidance exists for this broad patient group. The purpose of this survey is to establish what factors influence the decision to commence IGRT in adult patients, when diagnostic criteria for primary antibody deficiency are not fulfilled.Under the auspices of the United Kingdom Primary Immunodeficiency Network (UKPIN), a study group was established which circulated an online questionnaire to the consultant body across the UK and Ireland. Results provided a snapshot of the current clinical practice of 71% of consultant immunologists, from 30 centers.In order of importance, factors which influence the decision to commence IGRT include number of hospital admissions with infection, serum IgG level, bronchiectasis, radiologically proven pneumonia, number of positive sputum cultures, number of antibiotic courses, and results of immunization studies. The commonest test vaccine used was Pneumovax 23 with measurement of serotype-specific responses at 4 weeks, with a threshold of 0.35 mu g/ml in 2/3 of serotypes measured. Eighty-six percent of patients are treated with a trial of prophylactic antibiotics prior to consideration of IGRT. Efficacy of IGRT trial is assessed at between 6 and 12 months.There was consistency in clinical practice using a combination of clinical history, evidence of infections, and vaccination testing for diagnosis. However, there was some variation in the implementation of this practice, particularly in vaccine choice and assessment of response to vaccination.