Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency Publisher Pubmed



Pashangzadeh S¹ ; Delavari S^{1, 2} ; Moeini Shad T¹ ; Salami F^{1, 2} ; Rasouli SE^{1, 3} ; Yazdani R¹ ; Mahdaviani SA⁴ ; Nabavi M⁵ ; Aleyasin S⁶ ; Ahanchian H⁷ ; Jabbariazad F⁷ ; Chavoshzadeh Z⁸ ; Nazari F¹ ; Momen T⁹ Show All Authors
Source: Journal of Investigational Allergology and Clinical Immunology Published:2024

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. 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. © 2024 Esmon Publicidad.