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Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis
Seminars in Respiratory and Critical Care Medicine ( IF 3.2 ) Pub Date : 2021-07-14 , DOI: 10.1055/s-0041-1730893
Pamela J McShane 1
Affiliation  

Immunodeficiency represents a vast number of diseases and syndromes. Both primary and secondary forms of immunodeficiency are important contributors to the development of bronchiectasis. Primary immune deficiencies, in particular, are increasingly identified and defined as contributors. Specific immune deficiencies that are closely associated with bronchiectasis and as discussed in this article are common variable immunodeficiency, specific antibody deficiency, immunodeficiencies involving immunoglobulin E, DOCK8 immunodeficiency, phosphoglucomutase 3 deficiency, activated phosphoinositide 3-kinase delta syndrome, and X-linked agammaglobulinemia. Each of these primary immune deficiencies has unique nuances. Vigilance for these unique signs and symptoms is likely to improve recognition of specific immunodeficiency in the idiopathic bronchiectasis patient. Secondary forms of immunodeficiency occur as a result of a separate disease process. Graft versus host disease, malignancy, and human immunodeficiency virus are three classic examples discussed in this article. An awareness of the potential for these disease settings to lead to bronchiectasis is necessary to optimize patient care. With understanding and mindfulness toward the intricate relationship between bronchiectasis and immunodeficiency, there is an opportunity to elucidate pathophysiologic underpinnings between these two syndromes.



中文翻译:

支气管扩张症中常见的可变免疫缺陷和其他免疫缺陷综合征

免疫缺陷代表了大量的疾病和综合征。原发性和继发性免疫缺陷都是支气管扩张的重要因素。尤其是原发性免疫缺陷,越来越多地被识别和定义为贡献者。与支气管扩张密切相关且本文讨论的特异性免疫缺陷是常见的可变免疫缺陷、特异性抗体缺陷、涉及免疫球蛋白 E 的免疫缺陷、DOCK8 免疫缺陷、磷酸葡萄糖变位酶 3 缺陷、活化磷酸肌醇 3-激酶 δ 综合征和 X 连锁无丙种球蛋白血症。这些主要免疫缺陷中的每一个都有独特的细微差别。对这些独特的体征和症状保持警惕可能会提高对特发性支气管扩张患者特异性免疫缺陷的识别。免疫缺陷的继发性形式是由单独的疾病过程引起的。移植物抗宿主病、恶性肿瘤和人类免疫缺陷病毒是本文讨论的三个经典例子。有必要了解这些疾病环境导致支气管扩张的可能性,以优化患者护理。有了对支气管扩张和免疫缺陷之间复杂关系的理解和关注,就有机会阐明这两种综合征之间的病理生理学基础。和人类免疫缺陷病毒是本文讨论的三个经典例子。有必要了解这些疾病环境导致支气管扩张的可能性,以优化患者护理。有了对支气管扩张和免疫缺陷之间复杂关系的理解和关注,就有机会阐明这两种综合征之间的病理生理学基础。和人类免疫缺陷病毒是本文讨论的三个经典例子。有必要了解这些疾病环境导致支气管扩张的可能性,以优化患者护理。随着对支气管扩张和免疫缺陷之间复杂关系的理解和关注,有机会阐明这两种综合征之间的病理生理学基础。

更新日期:2021-07-15
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