Adult Common Variable Immunodeficiency

Abstract

Background: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia, defective antibody production and recurrent upper and lower respiratory tract infections. The diagnosis in adult patients is often thought to be rare, and thus misdiagnosis often occurs. A limited number of cases of adult-onset CVID have been reported in China, and the features of the syndrome remain unclear. The objective of this study was to describe the main characteristics of CVID, and evaluate the treatment of adult patients who present with CVID.

Materials and methods: This was a retrospective analysis of 8 patients with CVID from different departments in 1 center in China. Patients were diagnosed according to the diagnostic criteria of the European Society for Immunodeficiency Diseases. Demographics, clinical and immunological data from each patient were collected and a statistical analysis was undertaken.

Results: The mean age at diagnosis was 43 ± 13.7 years, whereas the mean duration of diagnostic delay was 10.5 years. The median total serum levels of immunoglobulin (Ig) G, IgA and IgM at diagnosis were 2.5 ± 0.59, 0.23 ± 0.05 and 0.17 ± 0.05g/L, respectively. A total of 7 patients also had a low CD4(+)/CD8(+) ratio. All patients presented with recurrent respiratory infections. Regular infusions of intravenous immunoglobulin every 3 weeks substantially reduced pneumonic episodes.

Conclusions: Diagnosis is often delayed in adult CVID. Pulmonary infections and diseases were the most frequent presentations at onset of the disease. Regular intravenous immunoglobulin infusions were beneficial in controlling recurrent infections.

Keywords: Adult; Common variable immunodeficiency; Infection.