Author:
Pashangzadeh S,Delavari S,Shad TM,Salami F,Rasouli SE,Yazdani R,Mahdaviani SA,Nabavi M,Aleyasin S,Ahanchian H,Azad FJ,Chavoshzadeh Z,Nazari F,Momen T,Sherkat R,Abolnezhadian F,Esmaeilzadeh H,Fallahpour M,Arshi S,Bemanian MH,Shokri S,Ebrahimi SS,Abolmolouki M,Farid AS,Rezaei A,Esmaeili M,Kalantari A,Sadeghi-Shabestari M,Shirkani A,Behniafard N,Khalili A,Eslamian MH,Cheraghi T,Shafie A,Tavakol M,Khoshkhui M,Iranparast S,Shamshiri M,Shahri MA,Khazaei R,Asadi M,Babaha F,Aghamohammadi A,Rezaei N,Abolhassani H
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, non-infectious complications are also a major challenge among CVID patients. Methods: All registered CVID patients in the national database were included in this retrospective cohort study. Patients were divided into two groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, non-infectious organ involvements, autoimmunity, and lymphoproliferative diseases were evaluated. Results: Among 387 enrolled patients, 66.4% were diagnosed with non-infectious complications; however, 33.6% had only 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 higher among patients with B-cell lymphopenia. Among organ involvement, 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 compared to other types of autoimmunity independent from the B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were slightly introduced as the most common type of malignancy. Meanwhile, the mortality rate was 24.5%, and respiratory failure and malignancies were reported as the most common cause of death in our patients without significant differences between the two groups. Conclusion: Considering that some of the non-infectious complications might be associated with B-cell lymphopenia, therefore, regular patient monitoring and follow-up along with proper medications (besides immunoglobulins replacement therapy) are highly recommended to prevent further sequels and increase the patients’ quality of life.
Subject
Immunology,Immunology and Allergy