CT Screening for Pulmonary Pathology in Common Variable Immunodeficiency Disorders and the Correlation with Clinical and Immunological Parameters

被引:54
|
作者
Maarschalk-Ellerbroek, L. J. [1 ]
de Jong, P. A. [2 ]
van Montfrans, J. M. [3 ]
Lammers, J. W. J. [4 ]
Bloem, A. C. [5 ]
Hoepelman, A. I. M. [1 ]
Ellerbroek, P. M. [1 ]
机构
[1] Univ Med Ctr Utrecht, Dept Internal Med & Infect Dis, NL-3508 GA Utrecht, Netherlands
[2] Univ Med Ctr Utrecht, Dept Radiol, NL-3508 GA Utrecht, Netherlands
[3] Univ Med Ctr Utrecht, Wilhelmina Childrens Hosp, Dept Pediat Immunol & Infect Dis, NL-3508 GA Utrecht, Netherlands
[4] Univ Med Ctr Utrecht, Dept Pulm Dis, NL-3508 GA Utrecht, Netherlands
[5] Univ Med Ctr Utrecht, Dept Immunol, NL-3508 GA Utrecht, Netherlands
关键词
Common variable immunodeficiency; thin slice CT scan; pulmonary function test; structural airway disease; interstitial lung disease; MEMORY B-CELLS; INTRAVENOUS IMMUNOGLOBULIN; COMPUTED-TOMOGRAPHY; IMMUNE-DEFICIENCY; DISEASE; ABNORMALITIES; ASSOCIATIONS; PNEUMONIA; FEATURES; HYPOGAMMAGLOBULINEMIA;
D O I
10.1007/s10875-014-0068-6
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Pulmonary disease is common in patients with common variable immunodeficiency disorders (CVID) and involves infections, chronic airway disease and interstitial lung disease. Chronic pulmonary disease is associated with excess morbidity and early mortality and therefore early detection and monitoring of progression is essential. Thin slice CT scan and pulmonary function were used to determine the prevalence and spectrum of chronic (pre-clinical) pulmonary disease in adult CVID patients regardless of symptoms. CT Scans were scored for airway abnormalities (AD) and interstitial lung disease (ILD). Other CVID related complications and B and T lymphocyte subsets were analyzed to identify patients at risk for pulmonary disease. Significant pulmonary abnormalities were detected in 24 of the 47 patients (51 %) consisting of AD in 30 % and ILD in 34 % of cases. In only 7 (29 %) of these 24 patients pulmonary function test proved abnormal. The presence of AD was correlated to (recurrent) lower respiratory tract infections despite IgG therapy. The presence of ILD was correlated to autoimmune disease and a reduction in the numbers of CD4 + T cells, na < ve CD4 + T cells, na < ve CD8 + T cells and memory B cells and lower IgG through levels over time. Preclinical signs of AD and ILD are common in CVID patients despite Ig therapy and do not correlate to pulmonary function testing. Patients at risk for ILD might be identified by the presence of autoimmunity or a deranged T cell pattern. Larger studies are needed to confirm these findings and to determine thresholds for the T lymphocyte subsets.
引用
收藏
页码:642 / 654
页数:13
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