期刊
JOURNAL OF CLINICAL IMMUNOLOGY
卷 41, 期 1, 页码 29-37出版社
SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10875-020-00854-y
关键词
22q11; 2; DiGeorge syndrome; lymphopenia; autoimmunity; asthma; infections
类别
资金
- National Institute of Allergy and Infectious Diseases (NIAID) [U24AI86837]
The study found that low absolute CD3+ T cell counts below 50% of age-adjusted normal values in patients with DiGeorge syndrome are associated with higher odds of autoimmunity and/or asthma.
Purpose DiGeorge syndrome has substantial heterogeneity with variable immune deficiency and dysregulation. Implicated immunopathology includes reduced thymic output and increased peripheral homeostatic proliferation with Th2 skewing and expansion of self-reactive cells. We hypothesized that T cell lymphopenia severity will be associated with higher odds of autoimmunity and/or asthma. Methods Using the US Immunodeficiency Network registry, we identified patients with 22q11.2 deletion (and/or TBX1). Initial absolute CD3+ T cell values were stratified: normal, 50-99% and below 50% of the lower limit of age-adjusted normal values. Patients with and without reported autoimmunity and asthma were compared using chi-square tests and multivariate logistic regression. Results Among 415 patients, autoimmunity was reported in 17 (4.1%), and asthma was reported in 28 (6.7%). Compared with those with no reported autoimmunity, patients with reported autoimmunity more frequently had low CD19+ B cells [3.3% (12/364) vs 28.6% (4/14);p = 0.002] and low IgG [6.2% (20/321) vs 29.4% (5/17);p = 0.005] levels. There were no statistically significant differences in other immune characteristics among those with and without reported asthma. Patients with absolute CD3 levels below 50% of age-adjusted normal values had higher odds of reported autoimmunity (n = 319, OR = 7.56, 95% CI = 1.58-36.17,p = 0.01) and reported asthma (n = 319, OR = 4.5, 95% CI = 1.06-18.93,p = 0.04) as compared with those with normal CD3 values, adjusted for age and low IgG. Conclusions Absolute CD3+ T cell counts below 50% of age-adjusted normal values may be associated with higher odds of autoimmunity and/or asthma in patients with DiGeorge syndrome and be potentially useful to identify higher-risk patients.
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