| Autor: |
S Pashangzadeh, S Delavari, TM Shad, F Salami, SE Rasouli, R Yazdani, SA Mahdaviani, M Nabavi, S Aleyasin, H Ahanchian, FJ Azad, Z Chavoshzadeh, F Nazari, T Momen, R Sherkat, F Abolnezhadian, H Esmaeilzadeh, M Fallahpour, S Arshi, MH Bemanian, S Shokri, SS Ebrahimi, M Abolmolouki, AS Farid, A Rezaei, M Esmaeili, A Kalantari, M Sadeghi-Shabestari, A Shirkani, N Behniafard, A Khalili, MH Eslamian, T Cheraghi, A Shafie, M Tavakol, M Khoshkhui, S Iranparast, M Shamshiri, MA Shahri, R Khazaei, M Asadi, F Babaha, A Aghamohammadi, N Rezaei, H Abolhassani |
| Rok vydání: |
2023 |
| Předmět: |
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| Zdroj: |
Journal of Investigational Allergy and Clinical Immunology. 33 |
| ISSN: |
1018-9068 |
| Popis: |
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. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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