Cardiac Complications in Patients with Common Variable Immune Deficiency: A Longitudinal Study
Background: The common variable immune deficiency (CVID) is known as the most prevalent symptomatic primary immune deficiency (PID) diseases, which is characterized by hypogammaglobulinemia with variable infectious and noninfectious manifestations. In this study, the researchers aimed to evaluate the frequency of cardiac disorders and investigate its association with other manifestations in CVID patients.
Method: A total of 337 CVID patients registered in the Iranian Primary Immunodeficiency Registry were evaluated in this study. The questionnaire was completed for all patients to collect the participants’ demographic data, clinical manifestations and laboratory finding. The analysis was performed between the two groups of the study including CVID patients with cardiac manifestation and those without it.
Results: The prevalence rate of cardiac manifestation was calculated to be 9.1%. pericardial and myocardial diseases and pulmonary hypertension were the most prevalent complications. CVID patients with a history of cardiac problem had significantly higher prevalence rates of otitis media, lymphoproliferative disorders, splenomegaly, hepatomegaly, failure to thrive and lower numbers of CD8+ T cells and CD19+ B cells compared to the patients without cardiac disorders. Notably, no significant differences were observed in immunoglobulins serum levels, CD3+ and CD4+ T cells between the patients with and without cardiac manifestation.
Conclusion: Regular echocardiographic evaluation and of CVID patients for cardiac complications especially for inflammatory cardiac disease, heart failure and pulmonary hypertension, is critical to reduce the risk of heart disease.
2. Knight AK, Cunningham-Rundles C. Inflammatory and autoimmune complications of common variable immune deficiency. Autoimmun Rev. 2006;5(2):156-9. 10.1016/j.autrev.2005.10.002.
3. Cunningham-Rundles C, Maglione PJ. Common variable immunodeficiency. J Allergy Clin Immunol. 2012;129(5):1425-6.e3.
4. Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, et al. Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management. J Investig Allergol Clin Immunol. 2020;30(1):14-34.
5. Human A, Murguia-Favela L, Benson L, Roifman I, Grunebaum E. Cardiovascular abnormalities in primary immunodeficiency diseases. Lympho Sign J. 2014;2(3):107-34.
6. Johnston SL, Hill SJ, Lock RJ, Dwight JF, Unsworth DJ, Gompels MM. Echocardiographic abnormalities in primary antibody deficiency. Postgrad Med J. 2004;80(942):214.
7. Ramzi N, Yazdani S, Talakoob H, Jamee M, Karim H, Azizi G. Acute pericarditis: A peculiar manifestation of common variable immune deficiency. Allergol Immunopathol (Madr). 2021;49(3):115-9.
8. Payandeh P, Khoshkhui M, Jabbari Azad F, Farid Hosseini R. A Patient With Common Variable Immunodeficiency and Pericardial Effusion: A Case Report and Review of Literature. J Pediatr Rev. 2019;7(3):177-80.
9. Laufs H, Nigrovic PA, Schneider LC, Oettgen H, Del NP, Moskowitz IP, et al. Giant cell myocarditis in a 12-year-old girl with common variable immunodeficiency. Mayo Clin Proc. 2002;77(1):92-6.
10. Kashef S, Moghtaderi M, Borzouee M, Rezaei N. Giant aneurysm of thoracic and proximal abdominal aorta in a patient with common variable immunodeficiency. Iran J Allergy Asthma Immunol. 2011;10(2):133-7.
11. Abolhassani H, Kiaee F, Tavakol M, Chavoshzadeh Z, Mahdaviani SA, Momen T, et al. Fourth Update on the Iranian National Registry of Primary Immunodeficiencies: Integration of Molecular Diagnosis. J Clin Immunol. 2018;38(7):816-32.
12. Diagnostic criteria of CVID: [Available from: https://esid.org/Working-Parties/Registry/Diagnosis-criteria.
13. Cooper LT, Jr. Myocarditis. N Engl J Med. 2009;360(15):1526-38.
14. Inamdar AA, Inamdar AC. Heart Failure: Diagnosis, Management and Utilization. J Clin Med. 2016;5(7):62.
15. Maganti K, Rigolin VH, Sarano ME, Bonow RO. Valvular heart disease: diagnosis and management. Mayo Clin Proc. 2010;85(5):483-500.
16. Adler Y, Charron P, Imazio M, Badano L, Barón-Esquivias G, Bogaert J, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: The Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC)Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015;36(42):2921-64.
17. Sun R, Liu M, Lu L, Zheng Y, Zhang P. Congenital Heart Disease: Causes, Diagnosis, Symptoms, and Treatments. Cell Biochem Biophys. 2015;72(3):857-60.
18. Fu DG. Cardiac Arrhythmias: Diagnosis, Symptoms, and Treatments. Cell Biochem Biophys. 2015;73(2):291-6.
19. Yaghi S, Novikov A, Trandafirescu T. Clinical update on pulmonary hypertension. J Investig Med. 2020;68(4):821-7.
20. Zeinaloo AA, Aghamohammadi A, Shabanian R, Salavati A, Abdollahzade S, Rezaei N, et al. Echocardiographic abnormalities and their correlation with bronchiectasis score in primary antibody deficiencies. J Cardiovas Med (MD). 2010;11(4):244-9.
21. Providência R, Botelho A, Quintal N, Costa M, Quaresma A, Lopes P, et al. Pulmonary hypertension in patients with ostium secundum atrial septal defect--is it related to echocardiographic complexity? Rev Port Cardiol. 2009;28(10):1087-96.
22. Cambray-Gutiérrez JC, Fernández-Muñoz MJ, Del Rivero-Hernández LG, López-Pérez P, Chávez-García AA, Segura-Méndez NH. Structural and functional heart diseases in adult patients with common variable immunodeficiency. Rev Alerg Mex. 2015;62(2):91-7.
23. Cooper LT, Baughman KL, Feldman AM, Frustaci A, Jessup M, Kuhl U, et al. The role of endomyocardial biopsy in the management of cardiovascular disease: a scientific statement from the American Heart Association, the American College of Cardiology, and the European Society of Cardiology. Endorsed by the Heart Failure Society of America and the Heart Failure Association of the European Society of Cardiology. J Am Coll Cardiol. 2007;50(19):1914-31.
24. Maglione PJ. Autoimmune and Lymphoproliferative Complications of Common Variable Immunodeficiency. Curr Allergy Asthma Rep. 2016;16(3):19.
25. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2019;123(5):454-60.
|Issue||Vol 4, No 2 (2021)|
|Common Variable Immune Deficiency Cardiac Disorders Clinical Manifestations Autoimmunity Pericardial Disease|
|Rights and permissions|
|This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.|