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      The clinical and immunological characteristics of common variable immunodeficiency in adults and older adults

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          Abstract

          OBJECTIVE

          The aim of this study was to determine the clinical and immunological characteristics of older adults with common variable immunodeficiency (CVID).

          METHODS

          Patients aged ≥18 years who were followed up with the diagnosis of CVID between 2015 and 2020 were included in the study. The patients were separated into two age groups according to the age at diagnosis: the adult group, aged 18-65 years (n=49) and the older adult group, aged ≥65 years (n=11).

          RESULTS

          Splenomegaly (55.1% vs. 9.1%, p=0.006), bronchiectasis (53.0% vs. 9.1%, p=0.008), and autoimmunity (42.8% vs. 9.1%, p=0.036) were determined to be more common in the adult group than in the older adults. A similar frequency of malignancy was seen in both groups (6.1% vs. 9.1%, p=0.721). There were significantly more patients with no comorbidity in the older adult group than in the adult group (45.5% vs. 16.3%, p=0.034). Serum IgG and IgA levels were determined to be significantly higher in the older adult group than in the adult group (p=0.001 for all). The CD19+ B-cell count at the time of diagnosis was determined to be lower and the CD19+CD27+IgD- switched memory B-cells and CD16+CD56+ natural killer cell counts were higher in the older adults than in the adult group (p=0.016, p=0.032, p=0.044, respectively).

          CONCLUSION

          Knowledge of clinical and immunological differences in older adult CVID patients may be of benefit in polyclinic follow-up and in respect of changes to be made to the treatment plan.

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          Most cited references28

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          International Consensus Document (ICON): Common Variable Immunodeficiency Disorders.

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            Morbidity and mortality in common variable immune deficiency over 4 decades.

            The demographics, immunologic parameters, medical complications, and mortality statistics from 473 subjects with common variable immune deficiency followed over 4 decades in New York were analyzed. Median immunoglobulin levels were IgG, 246 mg/dL; IgA, 8 mg/dL; and IgM, 21 mg/dL; 22.6% had an IgG less than 100 mg/dL. Males were diagnosed earlier (median age, 30 years) than females (median age, 33.5 years; P = .004). Ninety-four percent of patients had a history of infections; 68% also had noninfectious complications: hematologic or organ-specific autoimmunity, 28.6%; chronic lung disease, 28.5%; bronchiectasis, 11.2%; gastrointestinal inflammatory disease, 15.4%; malabsorption, 5.9%; granulomatous disease, 9.7%; liver diseases and hepatitis, 9.1%; lymphoma, 8.2%; or other cancers, 7.0%. Females had higher baseline serum IgM (P = .009) and were more likely to develop lymphoma (P = .04); 19.6% of patients died, a significantly shorter survival than age- and sex-matched population controls (P < .0001). Reduced survival was associated with age at diagnosis, lower baseline IgG, higher IgM, and fewer peripheral B cells. The risk of death was 11 times higher for patients with noninfectious complications (hazard ratio = 10.95; P < .0001). Mortality was associated with lymphoma, any form of hepatitis, functional or structural lung impairment, and gastrointestinal disease with or without malabsorption, but not with bronchiectasis, autoimmunity, other cancers, granulomatous disease, or previous splenectomy.
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              The Role of Immunosenescence in the Development of Age-Related Diseases.

              Aging is a complex phenomenon leading to numerous changes in the physiological systems of the body. One of the most important changes, called immunosenescence, occurs in the immune system. Immunosenescence covers changes in the innate and the adaptive immune systems and is associated with a low-grade inflammation called inflammaging. Aging, likely via inflammaging, is also associated with the emergence of chronic diseases including cardiovascular and neurodegenerative diseases, cancer, and diabetes mellitus type 2. The origin of this inflammaging is not known with certainty, but several concurrent contributing factors have been suggested, such as aging-associated changes in the innate and adaptive immune response, chronic antigenic stimulation, the appearance of endogenous macromolecular changes, and the presence of senescent cells exhibiting a senescence-associated secretory phenotype. A better understanding of the multiple biological phenomena leading to these diseases via the immunosenescence associated with inflammaging provides a powerful target for interventions to increase the healthspan of elderly subjects.
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                Author and article information

                Contributors
                Journal
                North Clin Istanb
                North Clin Istanb
                North Clin Istanb
                Northern Clinics of Istanbul
                Kare Publishing (Turkey )
                2148-4902
                2536-4553
                24 June 2024
                2024
                : 11
                : 3
                : 201-207
                Affiliations
                [1 ]Department of Clinical Immunology and Allergy, Necip Fazil City Hospital, Kahramanmaras, Turkiye
                [2 ]Division of Clinical Immunology and Allergy, Department of Internal Medicine, Necmettin Erbakan University Meram Faculty of Medicine, Konya, Turkiye
                [3 ]Department of Clinical Immunology and Allergy, Konya City Hospital, Konya, Turkiye
                Author notes
                Correspondence: Eray Yildiz, MD. Necip Fazil Sehir Hastanesi, Klinik Immunoloji ve Alerji Klinigi, Kahramanmaras, Turkiye. Tel: +90 344 228 23 47 e-mail: drerayyldz@ 123456gmail.com
                Article
                NCI-11-201
                10.14744/nci.2023.49699
                11237832
                fa569c28-05ba-4575-b405-2c8b18f27d03

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0)

                History
                : 25 July 2023
                : 16 August 2023
                Categories
                Original Article
                Immunology & Allergy

                common variable immunodeficiency,clinical phenotype,immunosenescence,older adults

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