Yan Chenxi, Qiu Junke, Pan Xiaohong, Huang Xiaoqin, Pan Lei, Wang Caihong, Mao Minjie
Zhejiang Provincial Hospital of Integrated Traditional Chinese and Western Medicine, Zhejiang, 310003, People's Republic of China.
J Inflamm Res. 2025 Jan 6;18:127-132. doi: 10.2147/JIR.S492482. eCollection 2025.
Patients with selective IgA deficiency could have various clinical presentations ranging from asymptomatic to severe respiratory or gastrointestinal tract infection, as well as autoimmune disease and allergic reactions. Selective IgA deficiency is relatively common in Caucasians, but it is rare in the Asian population, meaning it could be easily missed in the clinic. In this study, we report a 26-year-old man with a history of asthma and nephrotic syndrome. He presented with symptoms of pulmonary infection, and his condition quickly deteriorated to respiratory failure that required endotracheal intubation and mechanical ventilation. Sputum smear; sputum, blood, and bronchoalveolar lavage fluid culture; and metagenomic sequencing examination identified multiple mixed pathogens, including , and . Finally, he was diagnosed with selective IgA deficiency after a laboratory test detected an extremely low serum IgA level (<0.06 g/L). The patient died of septic shock and multiorgan failure despite aggressive management with a combination of antibiotics and supportive care. We report this case to remind clinicians about this rare disease in the Asian population. Patients with multisystem illnesses that are related to immune dysregulation, such as asthma or nephrotic syndrome, should be tested for immune system disorder. Rare and mixed pathogens should be considered during antibiotic selection in patients with selective IgA deficiency.
选择性IgA缺乏症患者的临床表现各异，从无症状到严重的呼吸道或胃肠道感染，以及自身免疫性疾病和过敏反应。选择性IgA缺乏症在白种人中相对常见，但在亚洲人群中较为罕见，这意味着在临床上很容易被漏诊。在本研究中，我们报告了一名26岁有哮喘和肾病综合征病史的男性。他出现了肺部感染症状，病情迅速恶化为呼吸衰竭，需要气管插管和机械通气。痰涂片、痰、血和支气管肺泡灌洗 fluid培养以及宏基因组测序检查发现了多种混合病原体，包括……。最后，实验室检查发现血清IgA水平极低（<0.06 g/L）后，他被诊断为选择性IgA缺乏症。尽管联合使用抗生素和支持治疗进行了积极管理，该患者仍死于感染性休克和多器官功能衰竭。我们报告此病例以提醒临床医生注意亚洲人群中的这种罕见疾病。患有与免疫失调相关的多系统疾病（如哮喘或肾病综合征）的患者应进行免疫系统疾病检测。在选择性IgA缺乏症患者选择抗生素时应考虑罕见和混合病原体。
