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. 2024 Jul;34(4):233-245.
doi: 10.18176/jiaci.0902.

Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency

S Pashangzadeh  1 , S Delavari  1   2 , T Moeini Shad  1 , F Salami  1   2 , S E Rasouli  1   3 , R Yazdani  1 , S A Mahdaviani  4 , M Nabavi  5 , S Aleyasin  6 , H Ahanchian  7 , F Jabbari-Azad  7 , Z Chavoshzadeh  8 , F Nazari  1 , T Momen  9 , R Sherkat  10 , F Abolnezhadian  11 , H Esmaeilzadeh  6 , M Fallahpour  5 , S Arshi  5 , M H Bemanian  5 , S Shokri  5 , S S Ebrahimi  12 , M Abolmolouki  1 , A S Farid  1 , A Rezaei  1 , M Esmaeili  1   2 , A Kalantari  13 , M Sadeghi-Shabestari  14 , A Shirkani  15 , N Behniafard  16 , A Khalili  17 , M H Eslamian  18 , T Cheraghi  19 , A Shafie  20 , M Tavakol  3 , M Khoshkhui  7 , S Iranparast  21 , M Shamshiri  21 , M A Shahri  1 , R Khazaei  11 , M Asadi  1 , F Babaha  1 , A Aghamohammadi  1 , N Rezaei  1   2 , H Abolhassani  1   22
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Free article

Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency

S Pashangzadeh et al. J Investig Allergol Clin Immunol. 2024 Jul.
Free article

Abstract

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.

Keywords: Autoimmunity; Common variable immunodeficiency; Immune dysregulation; Inborn errors of immunity; Malignancy; Primary immunodeficiency.

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