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支气管扩张症揭示的普通变异型免疫缺陷:一例报告

Common Variable Immunodeficiency Revealed by Bronchiectasis: A Case Report.

作者信息

Lakhal Mohamed, El Baroudi Touria, Rhazari Meriem, Gartini Sara, Thouil Afaf, Kouismi Hatim

机构信息

Department of Pulmonology, Research and Medical Sciences Laboratory, Centre Hospitalier Universitaire (CHU) Mohammed VI, Faculty of Medicine and Pharmacy, Mohammed I University, Oujda, MAR.

出版信息

Cureus. 2025 Apr 3;17(4):e81647. doi: 10.7759/cureus.81647. eCollection 2025 Apr.

Abstract

We report a case of a 38-year-old female with a past medical history of lymph node tuberculosis treated for six months in 2015, history of bronchiectasis from the past six years, and recurrent sinopulmonary infections, who was subsequently diagnosed to have common variable immunodeficiency (CVID). She had reduced levels of immunoglobulins during our diagnostic workup, after ruling out hematological malignancy and solid tumors. CVID is a highly heterogeneous group of disorders characterized by a primary defect in immunoglobulin production and an inability to mount a specific humoral response against exogenous antigens. The most frequently reported pulmonary manifestations of CVID are infectious pneumonias. Bronchiectasis, resulting from recurrent infections, is the third most common pulmonary manifestation observed in CVID patients, following pneumonia and bacterial bronchitis. The therapeutic management of CVID focuses on evaluating complications, with particular emphasis on the risks of bronchial dystrophy (bronchiectasis) and bronchial colonization by antibiotic-resistant pathogens. Respiratory physiotherapy is a key element in the management of bronchial suppuration. Treatment for CVID mainly consists of immunoglobulin replacement therapy, administered intravenously or subcutaneously, which must be given for life.

摘要

我们报告一例38岁女性病例,该患者有淋巴结结核病史,2015年接受了6个月的治疗,有6年支气管扩张病史,且反复发生鼻窦肺部感染,随后被诊断为普通变异型免疫缺陷(CVID)。在排除血液系统恶性肿瘤和实体瘤后,我们在诊断检查过程中发现她的免疫球蛋白水平降低。CVID是一组高度异质性疾病,其特征是免疫球蛋白产生存在原发性缺陷,且无法对外源性抗原产生特异性体液反应。CVID最常报告的肺部表现是感染性肺炎。反复感染导致的支气管扩张是CVID患者中观察到的第三常见肺部表现,仅次于肺炎和细菌性支气管炎。CVID的治疗管理重点在于评估并发症,尤其要关注支气管营养不良(支气管扩张)和抗生素耐药病原体支气管定植的风险。呼吸物理治疗是支气管化脓管理的关键要素。CVID的治疗主要包括静脉或皮下注射免疫球蛋白替代疗法,且必须终身进行。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dfa/12048885/a7842a633079/cureus-0017-00000081647-i01.jpg

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