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Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency Publisher Pubmed



Pashangzadeh S1 ; Delavari S1, 2 ; Moeini Shad T1 ; Salami F1, 2 ; Rasouli SE1, 3 ; Yazdani R1 ; Mahdaviani SA4 ; Nabavi M5 ; Aleyasin S6 ; Ahanchian H7 ; Jabbariazad F7 ; Chavoshzadeh Z8 ; Nazari F1 ; Momen T9 Show All Authors
Authors
  1. Pashangzadeh S1
  2. Delavari S1, 2
  3. Moeini Shad T1
  4. Salami F1, 2
  5. Rasouli SE1, 3
  6. Yazdani R1
  7. Mahdaviani SA4
  8. Nabavi M5
  9. Aleyasin S6
  10. Ahanchian H7
  11. Jabbariazad F7
  12. Chavoshzadeh Z8
  13. Nazari F1
  14. Momen T9
  15. Sherkat R10
  16. Abolnezhadian F11
  17. Esmaeilzadeh H6
  18. Fallahpour M5
  19. Arshi S5
  20. Bemanian MH5
  21. Shokri S5
  22. Ebrahimi SS12
  23. Abolmolouki M1
  24. Farid AS1
  25. Rezaei A1
  26. Esmaeili M1, 2
  27. Kalantari A13
  28. Sadeghishabestari M14
  29. Shirkani A15
  30. Behniafard N16
  31. Khalili A17
  32. Eslamian MH18
  33. Cheraghi T19
  34. Shafie A20
  35. Tavakol M3
  36. Khoshkhui M7
  37. Iranparast S21
  38. Shamshiri M21
  39. Shahri MA1
  40. Khazaei R11
  41. Asadi M1
  42. Babaha F1
  43. Aghamohammadi A1
  44. Rezaei N1, 2
  45. Abolhassani H1, 22
Show Affiliations
Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
  2. 2. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  3. 3. Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran
  4. 4. Pediatric Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
  5. 5. Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran
  6. 6. Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
  7. 7. Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
  8. 8. Pediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  9. 9. Department of Allergy and Clinical Immunology, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran
  10. 10. Immunodeficiency Diseases Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
  11. 11. Department of Pediatrics, Abuzar Children's Hospital, Ahvaz University of Medical Sciences, Ahvaz, Iran
  12. 12. Department of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran
  13. 13. Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran
  14. 14. Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran
  15. 15. Allergy and Clinical Immunology Department, Bushehr University of Medical Sciences, School of Medicine, Bushehr, Iran
  16. 16. Children Growth Disorder Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
  17. 17. Department of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
  18. 18. Department of Pediatrics, Hamedan University of Medical Sciences, Hamedan, Iran
  19. 19. Department of Pediatrics, 17 Shahrivar Children’s Hospital, Guilan University of Medical Sciences, Rasht, Iran
  20. 20. Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
  21. 21. Department of Immunology, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran
  22. 22. Division of Immunology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, Stockholm, Sweden

Source: Journal of Investigational Allergology and Clinical Immunology Published:2024


Abstract

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, noninfectious complications are a major challenge among CVID patients. 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. Conclusion: 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. © 2024 Esmon Publicidad.
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