- Author:
Lian-Jun LIN
;
Yu-Chuan WANG
;
Xin-Min LIU
1
Author Information
- Publication Type:Journal Article
- MeSH: Adolescent; Adult; Aged; Bronchiectasis; drug therapy; immunology; pathology; Child; Child, Preschool; China; Common Variable Immunodeficiency; drug therapy; immunology; pathology; Humans; Immunoglobulins; metabolism; Immunoglobulins, Intravenous; Middle Aged; Young Adult
- From: Chinese Medical Journal 2015;128(3):310-315
- CountryChina
- Language:English
-
Abstract:
BACKGROUNDCommon variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndromes. The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment.
METHODSClinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively.
RESULTSThe median age at onset was 11-year-old (range 4-51 years). The median age at diagnosis was 14.5-year-old (range 5-66 years). The average time of delay in diagnosis was 5.3 years (range 1-41 years). The most common main complaint was fever due to infections (35 cases, 87.5%). Pneumonia (28 cases, 70%) was the most common type of infections. Bronchiectasis was present in 6 patients (15%). Autoimmune disease was detected in 6 cases of CVID, and malignancy in 2 cases. The median total serum levels of IgG, IgA, and IgM at diagnosis were 1.07 g/L, 0.07 g/L, and 0.28 g/L, respectively. The percentages of CD3- /CD19 + B-cells were 1%-3.14%.
CONCLUSIONSInfection is the most frequent presentation of CVID. Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig) and lymphocyte subset analysis. Regular and sufficient substitution with Ig is recommended.