应对长期无症状免疫缺陷的管理复杂性。

IF 2.6 3区 医学 Q2 ALLERGY Allergy and asthma proceedings Pub Date : 2023-11-01 DOI:10.2500/aap.2023.44.230054
Katherine Park, Qing Wang, Rachel U Lee
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引用次数: 1

摘要

背景:常见可变免疫缺陷病(CVID)是一种与反复感染和非感染性结果相关的疾病,包括支气管扩张、肉芽肿性和淋巴细胞性间质性肺病(GLILD)、自身免疫性疾病、肠病和淋巴瘤。治疗包括开始替代免疫球蛋白(Ig),这是一项终身承诺。在Ig替代之前,CVID患者的预期寿命不到15年。随着Ig的替代,它已经改善到超过50年。在大多数情况下,患者会向有复发感染史的临床医生提出治疗建议。然而,对于无症状疾病患者,开始治疗的最佳时机可能很难确定。病例:我们报告了一例原本健康的男性,偶然诊断为CVID。结果:检查显示30多年来一直存在低丙种球蛋白血症。讨论:尽管Ig替代能成功减少感染,但也会带来许多副作用,给患者和医疗系统带来沉重的医疗负担。这也是一个很大的生活调整,可以极大地影响患者的生活质量。在军队中,免疫缺陷的诊断和每月静脉注射免疫球蛋白(IVIG)的需求可能对部署准备和患者的军事生涯不利。在开始Ig治疗之前,需要权衡风险和收益。
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Navigating the management complexity in long-term asymptomatic immunodeficiency.

Background: Common variable immunodeficiency disorder (CVID) is a condition associated with recurrent infections and non-infectious outcomes, including lung disease like bronchiectasis and granulomatous and lymphocytic interstitial lung diseases (GLILD), autoimmune disease, enteropathy, and lymphoma. Treatment involves initiation of replacement immunoglobulin (Ig), which is a lifelong commitment. Prior to Ig replacement, life expectancy for patients with CVID was less than 15 years. With replacement Ig, it has improved to over 50 years. In most cases, patients present to a clinician with a history of recurrent infections, and treatment is indicated. However, in patients with asymptomatic disease, the best timing to start treatment can be difficult to determine. Case: We present a case of an otherwise healthy male who had an incidental diagnosis of CVID. Results: Workup revealed hypogammaglobulinemia for over 30 year. Discussion: Though successful in reducing infections, Ig replacement can come with many side effects, as well as a heavy medical burden to the patient and the healthcare system. It is also a big life adjustment, and can greatly affect a patient's quality of life. In the military, a diagnosis of an immunodeficiency, and the need for monthly intravenous immunoglobulin (IVIG) can be detrimental to deployment readiness, and a patient's military career. Risks and benefits need to be weighed prior to initiating Ig therapy.

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来源期刊
CiteScore
5.70
自引率
35.70%
发文量
106
审稿时长
6-12 weeks
期刊介绍: Allergy & Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists. The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma. Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.
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