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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
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Authors Affiliations
  1. 1. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  2. 2. Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran
  3. 3. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  5. 5. Department of Rheumatology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran
  6. 6. Uro-Oncology Research Center, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Student Research Committee, Tabriz University of Medical Sciences, Tabriz, Iran
  8. 8. Immunology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
  9. 9. Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran
  10. 10. Department of Immunology, School of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran
  11. 11. Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran
  12. 12. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden

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
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