Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.
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Pashangzadeh S, Delavari S, Moeini Shad T, Salami F, Rasouli S E, Yazdani R, Mahdaviani S A, Nabavi M, Aleyasin S, Ahanchian H, Jabbari-Azad F, Chavoshzadeh Z, Nazari F, Momen T, Sherkat R, Abolnezhadian F, Esmaeilzadeh H, Fallahpour M, Arshi S, Bemanian M H, Shokri S, Ebrahimi S S, Abolmolouki M, Farid A S, Rezaei A, Esmaeili M, Kalantari A, Sadeghi-Shabestari M, Shirkani A, Behniafard N, Khalili A, Eslamian M H, Cheraghi T, Shafie A, Tavakol M, Khoshkhui M, Iranparast S, Shamshiri M, Shahri M A, Khazaei R, Asadi M, Babaha F, Aghamohammadi A, Rezaei N, Abolhassani H
Аннотация
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.