S Pashangzadeh, S Delavari, T Moeini Shad, F Salami, S E Rasouli, R Yazdani, S A Mahdaviani, M Nabavi, S Aleyasin, H Ahanchian, F Jabbari-Azad, Z Chavoshzadeh, F Nazari, T Momen, R Sherkat, F Abolnezhadian, H Esmaeilzadeh, M Fallahpour, S Arshi, M H Bemanian, S Shokri, S S Ebrahimi, M Abolmolouki, A S Farid, A Rezaei, M Esmaeili, A Kalantari, M Sadeghi-Shabestari, A Shirkani, N Behniafard, A Khalili, M H Eslamian, T Cheraghi, A Shafie, M Tavakol, M Khoshkhui, S Iranparast, M Shamshiri, M A Shahri, R Khazaei, M Asadi, F Babaha, A Aghamohammadi, N Rezaei, H Abolhassani
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Along with infectious complications, which have numerous consequences, noninfectious complications are a major challenge among CVID patients.</p><p><strong>Methods: </strong>All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated.</p><p><strong>Results: </strong>Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly more frequent among patients with B-cell lymphopenia. As for organ involvement, the dermatologic, endocrine, and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups.</p><p><strong>Conclusions: </strong>Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.</p>","PeriodicalId":50173,"journal":{"name":"Journal of Investigational Allergology and Clinical Immunology","volume":"34 4","pages":"233-245"},"PeriodicalIF":6.1000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.\",\"authors\":\"S Pashangzadeh, S Delavari, T Moeini Shad, F Salami, S E Rasouli, R Yazdani, S A Mahdaviani, M Nabavi, S Aleyasin, H Ahanchian, F Jabbari-Azad, Z Chavoshzadeh, F Nazari, T Momen, R Sherkat, F Abolnezhadian, H Esmaeilzadeh, M Fallahpour, S Arshi, M H Bemanian, S Shokri, S S Ebrahimi, M Abolmolouki, A S Farid, A Rezaei, M Esmaeili, A Kalantari, M Sadeghi-Shabestari, A Shirkani, N Behniafard, A Khalili, M H Eslamian, T Cheraghi, A Shafie, M Tavakol, M Khoshkhui, S Iranparast, M Shamshiri, M A Shahri, R Khazaei, M Asadi, F Babaha, A Aghamohammadi, N Rezaei, H Abolhassani\",\"doi\":\"10.18176/jiaci.0902\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. 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引用次数: 0
摘要
背景：常见变异性免疫缺陷病（CVID）被认为是人类先天性免疫错误中症状最严重的类型。感染性并发症会造成多种后果，而非感染性并发症也是 CVID 患者面临的一大挑战：这项回顾性队列研究纳入了国家数据库中登记的所有 CVID 患者。根据 B 细胞淋巴细胞减少症的存在将患者分为两组。研究人员对患者的人口统计学特征、实验室检查结果、非感染性器官受累情况、自身免疫和淋巴增生性疾病进行了评估：在 387 名登记的患者中，66.4% 被诊断为非感染性并发症，33.6% 被诊断为孤立的感染性表现。35.1%、24.3%和21.4%的患者出现肠病、自身免疫和淋巴增生性疾病。据报道，一些并发症，包括自身免疫和肝脾肿大，在B细胞淋巴细胞减少症患者中的发病率明显更高。在器官受累方面，B 细胞淋巴细胞减少症的 CVID 患者主要受累于皮肤、内分泌和肌肉骨骼系统。据报道，在自身免疫表现中，风湿病、血液病和胃肠道自身免疫的发生率高于其他与 B 细胞淋巴细胞减少症无关的自身免疫类型。此外，血液系统癌症，尤其是淋巴瘤，是最常见的恶性肿瘤类型。死亡率为24.5%，呼吸衰竭和恶性肿瘤是最常见的死亡原因，两组之间无明显差异：考虑到一些非感染性并发症可能与 B 细胞淋巴细胞减少症有关，因此强烈建议对患者进行定期监测，并通过适当的药物（除免疫球蛋白替代疗法外）进行随访，以防止后遗症的发生，提高患者的生活质量。
Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.
Background: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, noninfectious complications are a major challenge among CVID patients.
Methods: All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated.
Results: Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly more frequent among patients with B-cell lymphopenia. As for organ involvement, the dermatologic, endocrine, and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups.
Conclusions: Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.
期刊介绍：
The Journal of Investigational Allergology and Clinical Immunology (J Investig Allergol Clin Immunol) provides an attractive and very active forum for basic and clinical research in allergology and clinical immunology.Journal of Investigational Allergology and Clinical Immunology publishes original works, reviews, short communications and opinions.
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