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

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Abstract

Background

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.

Methods and Purpose

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.

Results

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, naïve CD4 + T cells, naïve CD8 + T cells and memory B cells and lower IgG through levels over time.

Conclusion

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.

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Grant Support

L.J. Maarschalk-Ellerbroek was supported by a non-restricted educational grant from Baxter Bioscience.

Author Contributions

L.J. Maarschalk-Ellerbroek: study concept and design, administrative acquisition of data, data entry, analysis and interpretation of data, statistical analysis, drafting of the manuscript and final approval of the version to be published.

P.A.de Jong: acquisition of data (evaluation and analyzing all CT scans results), critical revision of the manuscript for important intellectual content and final approval of the version to be published.

J. Montfrans: critical revision of the manuscript for important intellectual content and final approval of the version to be published.

J.W. Lammers: acquisition of data (evaluation and analyzing all pulmonary function tests results), critical revision of the manuscript for important intellectual content and final approval of the version to be published.

A.C. Bloem: acquisition of data (evaluation and analyzing all B- and T phenotyping results), critical revision of the manuscript for important intellectual content and final approval of the version to be published.

A.I.M. Hoepelman: study supervision, critical revision of the manuscript for important intellectual content and final approval of the version to be published.

P.M. Ellerbroek: study concept and design, study supervision, analysis and interpretation of data, statistical analysis, and drafting of the manuscript, critical revision of the manuscript for important intellectual content and final approval of the version to be published.

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Authors and Affiliations

  1. Department of Internal Medicine and Infectious Diseases, University Medical Centre of Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands

    L. J. Maarschalk-Ellerbroek, A. I. M. Hoepelman & P. M. Ellerbroek

  2. Department of Radiology, University Medical Centre of Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands

    P. A. de Jong

  3. Department of Pediatric Immunology and Infectious Diseases, University Medical Centre Utrecht/Wilhelmina Children’s Hospital, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands

    J. M. van Montfrans

  4. Department of Pulmonary Diseases, University Medical Centre Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands

    J. W. J. Lammers

  5. Department of Immunology, University Medical Center Utrecht, P.O. Box 85500, 3508, GA, Utrecht, The Netherlands

    A. C. Bloem

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  1. L. J. Maarschalk-Ellerbroek
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  3. J. M. van Montfrans
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Maarschalk-Ellerbroek, L.J., de Jong, P.A., van Montfrans, J.M. et al. CT Screening for Pulmonary Pathology in Common Variable Immunodeficiency Disorders and the Correlation with Clinical and Immunological Parameters. J Clin Immunol 34, 642–654 (2014). https://doi.org/10.1007/s10875-014-0068-6

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