Imaging of Bronchial Pathology in Antibody Deficiency: Data from the European Chest CT Group

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Authors	SCHUTZ Katharina ALECSANDRU Diana GRIMBACHER Bodo HADDOCK Jamanda BRUINING Annemarie DRIESSEN Gertjan DE VRIES Esther VAN HAGEN Peter M. HARTMANN Ieneke FRAIOLI Francesco MILITO Cinzia MITREVSKI Milica QUINTI Isabella SERRA Goffredo KELLEHER Peter LOEBINGER Michael LITZMAN Jiří POSTRÁNECKÁ Věra THON Vojtěch BABAR Judith CONDLIFFE Alison M. EXLEY Andrew KUMARARATNE Dinakantha SCREATON Nick JONES Alison BONDIONI Maria P. LOUGARIS Vassilios PLEBANI Alessandro SORESINA Annarosa SIRIGNANO Cesare SPADARO Giuseppe GALAL Nermeen GONZALEZ-GRANADO Luis I. DETTMER Sabine STIRLING Robert CHAPEL Helen LUCAS Mary PATEL Smita FARBER Claire-Michele MEYTS Isabelle BANERJEE Arpan K. HACKETT Scott HURST John R. WARNATZ Klaus GATHMANN Benjamin BAUMANN Ulrich
Year of publication	2019
Type	Article in Periodical
Magazine / Source	Journal of Clinical Immunology
MU Faculty or unit	Faculty of Medicine
Citation
Web	https://link.springer.com/article/10.1007%2Fs10875-018-0577-9
Doi	http://dx.doi.org/10.1007/s10875-018-0577-9
Keywords	Chest CT; CVID; primary antibody deficiency; bronchiectasis; bronchial pathology
Description	Studies of chest computed tomography (CT) in patients with primary antibody deficiency syndromes (ADS) suggest a broad range of bronchial pathology. However, there are as yet no multicentre studies to assess the variety of bronchial pathology in this patient group. One of the underlying reasons is the lack of a consensus methodology, a prerequisite to jointly document chest CT findings. We aimed to establish an international platform for the evaluation of bronchial pathology as assessed by chest CT and to describe the range of bronchial pathologies in patients with antibody deficiency. Ffteen immunodeficiency centres from 9 countries evaluated chest CT scans of patients with ADS using a predefined list of potential findings including an extent score for bronchiectasis. Data of 282 patients with ADS were collected. Patients with common variable immunodeficiency disorders (CVID) comprised the largest subgroup (232 patients, 82.3%). Eighty percent of CVID patients had radiological evidence of bronchial pathology including bronchiectasis in 61%, bronchial wall thickening in 44% and mucus plugging in 29%. Bronchiectasis was detected in 44% of CVID patients aged less than 20years. Cough was a better predictor for bronchiectasis than spirometry values. Delay of diagnosis as well as duration of disease correlated positively with presence of bronchiectasis. The use of consensus diagnostic criteria and a pre-defined list of bronchial pathologies allows for comparison of chest CT data in multicentre studies. Our data suggest a high prevalence of bronchial pathology in CVID due to late diagnosis or duration of disease.
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