Clinical Features of Immunological Dysregulation in Common Variable Immunodeficiency in Iran

Abstract

Background: Common Variable Immune Deficiency (CVID) is a primary immunodeficiency with significant clinical manifestations. The aim of this study was to evaluate the clinical and immunological characteristics of CVID patients in Isfahan city, Iran. Methods: Data of 25 CVID Patients were collected from referrals to alzahra hospital and the Acquired Immunodeficiency Research Center, Isfahan University of Medical Sciences between 2007 and 2013. The patients were diagnosed according to the Immunodeficiency Disease criteria and all patients re-ceived Intravenous immunoglobulin (IVIG) as a fundamental part of the treatment at a mean dose of 500 mg/kg. Results: The age at onset of disease was between 4 to 37 years and diagnosis was between 2 to 39 years. The median levels of IgG, IgM, and IgA were 36.75, 4.77 and1.05 mg/dL respectively. The per-centage of CD19+ B cells, CD20+, CD3+, CD4+, CD8+, CD16+CD56+ cells was 8.35%, 8%, 80.88%, 28.72%, 38.88%, and 11.08% respectively. Sinusitis (79%), pneumonia (85%) and acute otitis media (40%) were the most common manifestations. In addition, Bronchiectasis was seen in about 25% and Autoimmunity (Thrombocytopenia, RA) was found in 33% of cases. Allergic symptoms were present in 8% of patients. Conclusion: CVID patients can present with a wide range of manifestations such as infections, immu-nological dysfunctions (autoimmunity), allergy and malignancy. A variety of defects in both humoral and cell-mediated immune responses can occur in CVID patients.