Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency

被引：4

作者：

Pashangzadeh, S. ^{[1
]}

Delavari, S. ^{[1
,2
]}

Shad, T. Moeini ^{[1
]}

Salami, F. ^{[1
,2
]}

Rasouli, S. E. ^{[1
,3
]}

Yazdani, R. ^{[1
]}

Mahdaviani, S. A. ^{[4
]}

Nabavi, M. ^{[5
]}

Aleyasin, S. ^{[6
]}

Ahanchian, H. ^{[7
]}

Jabbari-Azad, F. ^{[7
]}

Chavoshzadeh, Z. ^{[8
]}

Nazari, F. ^{[1
]}

Momen, T. ^{[9
]}

Sherkat, R. ^{[10
]}

Abolnezhadian, F. ^{[11
]}

Esmaeilzadeh, H. ^{[6
]}

Fallahpour, M. ^{[6
]}

Arshi, S. ^{[5
]}

Bemanian, M. H. ^{[5
]}

Shokri, S. ^{[5
]}

Ebrahimi, S. S. ^{[12
]}

Abolmolouki, M. ^{[1
]}

Farid, A. S. ^{[1
]}

Rezaei, A. ^{[1
]}

Esmaeili, M. ^{[1
,2
]}

Kalantari, A. ^{[13
]}

Sadeghi-Shabestari, M. ^{[14
]}

Shirkani, A. ^{[15
]}

Behniafard, N. ^{[16
]}

Khalili, A. ^{[17
]}

Eslamian, M. H. ^{[18
]}

Cheraghi, T. ^{[19
]}

Shafie, A. ^{[20
]}

Tavakol, M. ^{[3
]}

Khoshkhui, M. ^{[7
]}

Iranparast, S. ^{[21
]}

Shamshiri, M. ^{[21
]}

Shahri, M. A. ^{[1
]}

Khazaei, R. ^{[11
]}

Asadi, M. ^{[1
]}

Babaha, F. ^{[1
]}

Aghamohammadi, A. ^{[1
]}

Rezaei, N. ^{[1
,2
]}

Abolhassani, H. ^{[1
,22
]}

机构：

[1] Univ Tehran Med Sci, Res Ctr Immunodeficiencies, Pediat Ctr Excellence, Childrens Med Ctr, Tehran, Iran

[2] Universal Sci Educ & Res Network USERN, Primary Immunodeficiency Dis Network PIDNet, Tehran, Iran

[3] Alborz Univ Med Sci, Noncommunicable Dis Res Ctr, Karaj, Iran

[4] Shahid Beheshti Univ Med Sci, Natl Res Inst TB & Lung Dis NRITLD, Pediat Resp Dis Res Ctr, Tehran, Iran

[5] Iran Univ Med Sci, Rasool E Akram Hosp, Dept Allergy & Clin Immunol, Tehran, Iran

[6] Shiraz Univ Med Sci, Allergy Res Ctr, Shiraz, Iran

[7] Mashhad Univ Med Sci, Allergy Res Ctr, Mashhad, Iran

[8] Shahid Beheshti Univ Med Sci, Mofid Childrens Hosp, Pediat Infect Res Ctr, Tehran, Iran

[9] Isfahan Univ Med Sci, Res Inst Primordial Prevent Noncommunicable Dis, Dept Allergy & Clin Immunol, Child Growth & Dev Res Ctr, Esfahan, Iran

[10] Isfahan Univ Med Sci, Immunodeficiency Dis Res Ctr, Esfahan, Iran

[11] Ahvaz Jundishapur Univ Med Sci, Abuzar Childrens Hosp, Dept Pediat, Ahvaz, Iran

[12] Kerman Univ Med Sci, Dept Immunol & Allergy, Kerman, Iran

[13] Univ Tehran Med Sci, Imam Khomeini Hosp, Dept Immunol & Allergy, Tehran, Iran

[14] Tabriz Univ Med Sci, Dept Immunol & Allergy, Tabriz, Iran

[15] Bushehr Univ Med Sci, Sch Med, Allergy & Clin Immunol Dept, Bushehr, Iran

[16] Shahid Sadoughi Univ Med Sci, Children Growth Disorder Res Ctr, Yazd, Iran

[17] Shahid Sadoughi Univ Med Sci, Dept Pediat, Yazd, Iran

[18] Hamedan Univ Med Sci, Dept Pediat, Hamadan, Iran

[19] Guilan Univ Med Sci, 17 Shahrivar Childrens Hosp, Dept Pediat, Rasht, Iran

[20] Univ Tehran Med Sci, Bahrami Hosp, Dept Immunol, Tehran, Iran

[21] Ahvaz Jundishapur Univ Med Sci, Fac Med, Dept Immunol, Ahvaz, Iran

[22] Karolinska Inst, Dept Med Biochem & Biophys, Div Immunol, Stockholm, Sweden

来源：

JOURNAL OF INVESTIGATIONAL ALLERGOLOGY AND CLINICAL IMMUNOLOGY | 2024年 / 34卷 / 04期

关键词：

Primary immunodeficiency; Inborn errors of immunity; Common variable immunodeficiency.Autoimmunity; immunodeficiency.Autoimmunity; Malignancy; Immune dysregulation; REGULATORY T-CELLS; IGA DEFICIENCY; AUTOIMMUNITY; DISORDERS; SUBSETS; COHORT; LYMPHOCYTES; PHENOTYPE; CHILDREN; CRITERIA;

D O I：

10.18176/jiaci.0902

中图分类号：

R392 [医学免疫学];

学科分类号：

100102 ;

摘要：

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. Conclusion: 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.

引用

页码：233 / 245

页数：13

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