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 ± 0.05 g/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.
Key Indexing Terms
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Article info
Footnotes
☆This work was supported by the Education Department of Shandong Province research grant (Project no. J10LF78) and the Qingdao Science and Technology Bureau research grant (Project No. 11-2-4-2-(13)-jch) to Dr Jing Dong.
☆☆The authors have no financial or other conflicts of interest to disclose.
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© 2016 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
