%0 Journal Article
%T Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.
%A Pashangzadeh S
%A Delavari S
%A Moeini Shad T
%A Salami F
%A Rasouli SE
%A Yazdani R
%A Mahdaviani SA
%A Nabavi M
%A Aleyasin S
%A Ahanchian H
%A Jabbari-Azad F
%A Chavoshzadeh Z
%A Nazari F
%A Momen T
%A Sherkat R
%A Abolnezhadian F
%A Esmaeilzadeh H
%A Fallahpour M
%A Arshi S
%A Bemanian MH
%A Shokri S
%A Ebrahimi SS
%A Abolmolouki M
%A Farid AS
%A Rezaei A
%A Esmaeili M
%A Kalantari A
%A Sadeghi-Shabestari M
%A Shirkani A
%A Behniafard N
%A Khalili A
%A Eslamian MH
%A Cheraghi T
%A Shafie A
%A Tavakol M
%A Khoshkhui M
%A Iranparast S
%A Shamshiri M
%A Shahri MA
%A Khazaei R
%A Asadi M
%A Babaha F
%A Aghamohammadi A
%A Rezaei N
%A Abolhassani H
%J J Investig Allergol Clin Immunol
%V 34
%N 4
%D 2024 Jul
%M 39077769
%F 8.185
%R 10.18176/jiaci.0902
%X <B>BACKGROUND: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.