Tehran University of Medical Sciences

Science Communicator Platform

Stay connected! Follow us on X network (Twitter):
Share By
Rheumatologic Complications in a Cohort of 227 Patients With Common Variable Immunodeficiency Publisher Pubmed



Azizi G1, 2, 3, 4 ; Kiaee F3, 4 ; Hedayat E3, 4 ; Yazdani R3 ; Dolatshahi E5 ; Alinia T3 ; Sharifi L6 ; Mohammadi H7, 8 ; Kavosi H9 ; Jadidiniaragh F8, 10 ; Ziaee V11 ; Abolhassani H3, 4, 12 ; Aghamohammadi A3, 4
Authors

Source: Scandinavian Journal of Immunology Published:2018


Abstract

Common variable immunodeficiency (CVID) is the most prevalent symptomatic type of human primary immunodeficiency diseases (PID). Clinically, CVID is characterized by increased susceptibility to infections and a wide variety of autoimmune and rheumatologic disorders. All patients with CVID registered in Iranian PID Registry (IPIDR) were enrolled in this retrospective cohort study. We investigated the frequency of rheumatologic diseases and its association with immunological and clinical phenotypes in patients with CVID. A total of 227 patients with CVID were enrolled in this study. The prevalence of rheumatologic disorders was 10.1% with a higher frequency in women than men. Most common rheumatologic manifestations were juvenile idiopathic arthritis (JIA) and adult rheumatoid arthritis (RA) followed by juvenile spondyloarthritis (JSpA) and undifferentiated inflammatory arthritis (UIA). Septic arthritis in patients with CVID with a history of RA and JIA was higher than patients without rheumatologic complication. Patients with CVID with a history of autoimmunity (both rheumatologic and non-rheumatologic autoimmunity) had lower regulatory T cells counts in comparison with patients without autoimmune disorders. There was an association between defect in specific antibody responses and negative serologic test results in patients with rheumatologic manifestations. JIA, RA, JSpA and UIA are the most frequent rheumatologic disorders in patients with CVID. Due to antibody deficiency, serologic tests may be negative in these patients. Therefore, these conditions pose significant diagnostic and therapeutic challenges for immunologists and rheumatologists in charge of the care for these patients. © 2018 The Foundation for the Scandinavian Journal of Immunology
Related Docs
View other Related Docs
1. Autoimmunity and Its Association With Regulatory T Cells and B Cell Subsets in Patients With Common Variable Immunodeficiency, Allergologia et Immunopathologia (2018)
2. Autoimmunity in Common Variable Immunodeficiency: Epidemiology, Pathophysiology and Management, Expert Review of Clinical Immunology (2017)
3. The Clinical and Immunological Features of Patients With Primary Antibody Deficiencies, Endocrine# Metabolic and Immune Disorders - Drug Targets (2018)
Experts (# of related papers)
View all Related Experts
Nima Rezaei (6)
Laleh Sharifi (4)
Other Related Docs
4. Autoimmune Disorders Associated With Common Variable Immunodeficiency: Prediction, Diagnosis, and Treatment, Expert Review of Clinical Immunology (2022)
5. Autoimmunity in Common Variable Immunodeficiency: A Systematic Review and Meta-Analysis, Expert Review of Clinical Immunology (2020)
6. Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management, Journal of Investigational Allergology and Clinical Immunology (2020)
7. A Comparison of Clinical and Immunologic Phenotypes in Familial and Sporadic Forms of Common Variable Immunodeficiency, Scandinavian Journal of Immunology (2017)
8. Approach to the Management of Autoimmunity in Primary Immunodeficiency, Scandinavian Journal of Immunology (2017)
9. Circulating Helper T-Cell Subsets and Regulatory T Cells in Patients With Common Variable Immunodeficiency Without Known Monogenic Disease, Journal of Investigational Allergology and Clinical Immunology (2018)
10. The Profile of Il-4, Il-5, Il-10 and Gata3 in Patients With Lrba Deficiency and Cvid With No Known Monogenic Disease: Association With Disease Severity, Allergologia et Immunopathologia (2019)
11. Role of Rare Immune Cells in Common Variable Immunodeficiency, Pediatric Allergy and Immunology (2022)
12. Autoimmunity in Primary Antibody Deficiencies, International Archives of Allergy and Immunology (2017)
13. G2-Lymphocyte Chromosomal Radiosensitivity in Patients With Lps Responsive Beige-Like Anchor Protein (Lrba) Deficiency, International Journal of Radiation Biology (2019)
14. Autoimmunity in a Cohort of 471 Patients With Primary Antibody Deficiencies, Expert Review of Clinical Immunology (2017)
15. Comparison of Clinical and Immunological Features and Mortality in Common Variable Immunodeficiency and Agammaglobulinemia Patients, Immunology Letters (2019)
16. Mannose-Binding Lectin Protein Deficiency Among Patients With Primary Immunodeficiency Disease Receiving Ivig Therapy, Endocrine# Metabolic and Immune Disorders - Drug Targets (2018)
17. A Review on Defects of Dendritic Cells in Common Variable Immunodeficiency, Endocrine# Metabolic and Immune Disorders - Drug Targets (2017)
18. Lymphocytes Subsets in Correlation With Clinical Profile in Cvid Patients Without Monogenic Defects, Expert Review of Clinical Immunology (2021)
19. Increased Irf4 Expression in Isolated B Cells From Common Variable Immunodeficiency (Cvid) Patients, Allergologia et Immunopathologia (2019)
20. Increased Expression of B Lymphocyte Induced Maturation Protein 1 (Blimp1) in Patients With Common Variable Immunodeficiency (Cvid), Iranian Journal of Allergy# Asthma and Immunology (2020)