Research Article

Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience

Volume: 3 Number: 1 January 29, 2023
Zeynep Yüksel *, Ahmet Zafer Calıskaner
EN

Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience

Abstract

Objectives: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by immunoglobulin production defect. Our study aimed to create awareness of primary immunodeficiency in adult patients, establish standard approaches for clinical follow-up of CVID patients, and reveal the clinical characteristics of CVID patients in our region. Method: The study was conducted in patients with diagnosed and newly diagnosed CVID. The demographic and clinical characteristics of the patients and their treatment data were analyzed retrospectively and prospectively. Results: Thirteen of our patients were female and 12 were male. The mean age at diagnosis of the patients was 30.32 (2-57) and the mean delay in diagnosis was 9.32 months (0-30). The most common clinical finding of our patients at the time of admission was an infection. Among the infections identified, 3 patients had URTI, 19 had LRTI, and 2 had gastroenteritis. In 16 of our patients, bronchiectasis was detected at the time of diagnosis, and in 1 during the follow-up period. In the examinations performed in terms of organomegaly, splenomegaly was found in 11 patients and hepatomegaly was found in 8 patients. When patients were screened for autoimmune disease, ITP and celiac were found in 2 patients at the beginning, while autoimmune thyroiditis was developed in 1 patient and SLE in 1 patient during follow-up. Our patients were given IVIG treatment at regular intervals. The number of reactions seen in a total of 421 IVIG infusions was two. Conclusion: Primary immunodeficiencies should definitely be considered in patients with recurrent infections and resistance to antibiotic therapy. Patients should be followed according to established follow-up and treatment protocols in order to reduce and diagnose complications.

Keywords

CVID, primary immunodeficiency, follow-up and treatment protocols

References

  1. Schroeder, HW, Jr. “Genetics of IgA deficiency and common variable immunodeficiency.” Clin Rev Allergy Immunol 2000;19(2): 127-140.
  2. Notarangelo, LD, A Fischer, RS Geha, JL Casanova, H Chapel, ME Conley, et al. “Primary immunodeficiencies: 2009 update.” J Allergy Clin Immunol 2009;124(6): 1161-1178.
  3. Conley, ME, J Rohrer, L Rapalus, EC Boylin and Y Minegishi. “Defects in early Bcell development: comparing the consequences of abnormalities in pre-BCR signaling in the human and the mouse.” Immunol Rev 2000;178: 75-90.
  4. Hammarstrom, L, I Vorechovsky and D Webster. “Selective IgA deficiency (SIgAD) and common variable immunodeficiency (CVID).” Clin Exp Immunol 2000;120(2): 225-231.
  5. Chapel, H, M Lucas, M Lee, J Bjorkander, D Webster, B Grimbacher, et al. “Common variable immunodeficiency disorders: division into distinct clinical phenotypes.” Blood 2008;112(2): 277-286.
  6. Quinti, I, A Soresina, G Spadaro, S Martino, S Donnanno, C Agostini, et al. “Longterm follow-up and outcome of a large cohort of patients with common variable immunodeficiency.” J Clin Immunol 2007;27(3): 308-316.
  7. Ardeniz, O, CB Avci, A Sin, G Ozgen, F Gunsar, N Mete, et al. “Vitamin D deficiency in the absence of enteropathy in three cases with common variable immunodeficiency.” Int Arch Allergy Immunol 2008;147(1): 74-83.
  8. Urschel, S, L Kayikci and U Wintergerst. “Common variable immundeficiency disorders in children: delayed diagnosis despite typical clinical presentation.” J Pediatr 2009;(154): 888.
  9. Conley, ME, LD Notarangelo and A Etzioni. “Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies).” Clin Immunol 1999;93(3): 190-197.
  10. Cunningham-Rundles, C and C Bodian. “Common variable immunodeficiency: clinical and immunological features of 248 patients.” Clin Immunol 1999;92(1): 34-48.
APA
Yüksel, Z., & Calıskaner, A. Z. (2023). Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience. DAHUDER Medical Journal, 3(1), 13-19. https://doi.org/10.56016/dahudermj.1225606
AMA
1.Yüksel Z, Calıskaner AZ. Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience. DAHUDER MJ. 2023;3(1):13-19. doi:10.56016/dahudermj.1225606
Chicago
Yüksel, Zeynep, and Ahmet Zafer Calıskaner. 2023. “Follow-up and Treatment of Patients With Common Variable Immune Deficiency: A Single-Center Experience”. DAHUDER Medical Journal 3 (1): 13-19. https://doi.org/10.56016/dahudermj.1225606.
EndNote
Yüksel Z, Calıskaner AZ (January 1, 2023) Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience. DAHUDER Medical Journal 3 1 13–19.
IEEE
[1]Z. Yüksel and A. Z. Calıskaner, “Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience”, DAHUDER MJ, vol. 3, no. 1, pp. 13–19, Jan. 2023, doi: 10.56016/dahudermj.1225606.
ISNAD
Yüksel, Zeynep - Calıskaner, Ahmet Zafer. “Follow-up and Treatment of Patients With Common Variable Immune Deficiency: A Single-Center Experience”. DAHUDER Medical Journal 3/1 (January 1, 2023): 13-19. https://doi.org/10.56016/dahudermj.1225606.
JAMA
1.Yüksel Z, Calıskaner AZ. Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience. DAHUDER MJ. 2023;3:13–19.
MLA
Yüksel, Zeynep, and Ahmet Zafer Calıskaner. “Follow-up and Treatment of Patients With Common Variable Immune Deficiency: A Single-Center Experience”. DAHUDER Medical Journal, vol. 3, no. 1, Jan. 2023, pp. 13-19, doi:10.56016/dahudermj.1225606.
Vancouver
1.Zeynep Yüksel, Ahmet Zafer Calıskaner. Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience. DAHUDER MJ. 2023 Jan. 1;3(1):13-9. doi:10.56016/dahudermj.1225606