Clinical and Immunological Features of 65 Iranian Patients with Common Variable Immunodeficiency

TLDR

Common variable immunodeficiency is a primary immunodeficiency marked by low immunoglobulins and recurrent bacterial infections. The study reviewed records of 65 CVID patients (37 male, 28 female) aged 24–537 months over 221 patient‑years of follow‑up. Among the cohort, 11 deaths and 7 lost to follow‑up occurred; median diagnostic delay was 60 months, baseline IgG, IgM, and IgA were 120, 10, and 0 mg/dl, and recurrent infections—especially pneumonia, diarrhea, sinusitis, and otitis media—were common at onset and during follow‑up, underscoring the need to consider CVID in patients with recurrent infections and hypogammaglobulinemia.

Abstract

ABSTRACT Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by hypogammaglobulinemia and recurrent bacterial infections. The records of 65 patients with CVID (37 males and 28 females) in the age range of 24 to 537 months were reviewed. By the year 2003, 11 patients had died and seven patients could not be located. The total follow-up period was 221 patient-years. The median diagnostic delay (time between onset and diagnosis) in our patient group was 60 months. At the time of diagnosis, the baseline serum immunoglobulin G (IgG), IgM, and IgA levels were below the level normal for the patients' age; the medians for this group were 120, 10, and 0 mg/dl, respectively. All of the patients presented with infectious diseases at the time of onset, the most common of which were otitis media, diarrhea, pneumonia, and sinusitis. Acute and recurrent infections were also found in almost all of the patients, particularly involving respiratory and gastrointestinal systems. The most common infections, before diagnosis and during follow-up, were pneumonia, acute diarrhea, acute sinusitis, and otitis media. CVID should be considered in any patient with a history of recurrent infections and decreased levels of all serum immunoglobulin isotypes.