Pulmonary Manifestations of Primary Humoral Deficiencies

IF 2.1 4区 医学 Q3 RESPIRATORY SYSTEM Canadian respiratory journal Pub Date : 2022-04-10 DOI:10.1155/2022/7140919
A. Casal, V. Riveiro, J. Suárez-Antelo, L. Ferreiro, N. Rodríguez-Núñez, A. Lama, M. Toubes, L. Valdés
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引用次数: 2

Abstract

Primary immunodeficiencies are a group of conditions characterized by developmental or functional alterations in the immune system caused by hereditary genetic defects. Primary immunodeficiencies may affect either the innate or the adaptive (humoral and cellular) immune system. Pulmonary complications in primary humoral deficiencies are frequent and varied and are associated with high morbidity and mortality rates. The types of complications include bronchiectasis secondary to recurrent respiratory infections and interstitial pulmonary involvement, which can be associated with autoimmune cytopenias, lymphoproliferation, and a range of immunological manifestations. Early detection is key to timely management. Immunoglobulin replacement therapy reduces the severity of disease, the frequency of exacerbations, and hospital admissions in some primary humoral deficiencies. Therefore, the presence of pulmonary disease with concomitant infectious and/or autoimmune complications should raise suspicion of primary humoral deficiencies and warrants a request for immunoglobulin determination in blood. Once diagnosis is confirmed; early immunoglobulin replacement therapy will improve the course of the disease. Further studies are needed to better understand the pathogenesis of pulmonary disease related to primary humoral deficiencies and favor the development of targeted therapies that improve the prognosis of patients.
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原发性体液缺乏的肺部表现
原发性免疫缺陷是一组由遗传性遗传缺陷引起的免疫系统发育或功能改变的疾病。原发性免疫缺陷可能影响先天或适应性(体液和细胞)免疫系统。原发性体液缺乏症的肺部并发症是常见和多样的,并与高发病率和死亡率相关。并发症类型包括继发于复发性呼吸道感染的支气管扩张症和肺间质受累,这可能与自身免疫性细胞减少、淋巴增殖和一系列免疫表现有关。早期发现是及时管理的关键。免疫球蛋白替代疗法可降低某些原发性体液缺乏症的疾病严重程度、恶化频率和住院率。因此,肺部疾病伴发感染性和/或自身免疫性并发症应引起对原发性体液缺乏的怀疑,并有必要要求在血液中测定免疫球蛋白。一旦确诊;早期免疫球蛋白替代治疗将改善疾病的进程。需要进一步的研究来更好地了解与原发性体液缺乏相关的肺部疾病的发病机制,并有利于开发改善患者预后的靶向疗法。
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来源期刊
Canadian respiratory journal
Canadian respiratory journal 医学-呼吸系统
CiteScore
4.20
自引率
0.00%
发文量
61
审稿时长
6-12 weeks
期刊介绍: Canadian Respiratory Journal is a peer-reviewed, Open Access journal that aims to provide a multidisciplinary forum for research in all areas of respiratory medicine. The journal publishes original research articles, review articles, and clinical studies related to asthma, allergy, COPD, non-invasive ventilation, therapeutic intervention, lung cancer, airway and lung infections, as well as any other respiratory diseases.
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