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

Katharina Schütz, Diana Alecsandru, Bodo Grimbacher, Jamanda Haddock, Annemarie Bruining, Gertjan Driessen, Esther de Vries, Peter M. van Hagen, Ieneke Hartmann, Francesco Fraioli, Cinzia Milito, Milica Mitrevski, Isabella Quinti, Goffredo Serra, Peter Kelleher, Michael Loebinger, Jiri Litzman, Vera Postranecka, Vojtech Thon, Judith BabarAlison M. Condliffe, Andrew Exley, Dinakantha Kumararatne, Nick Screaton, Alison Jones, Maria P. Bondioni, Vassilios Lougaris, Alessandro Plebani, Annarosa Soresina, Cesare Sirignano, Giuseppe Spadaro, Nermeen Galal, Luis I. Gonzalez-Granado, Sabine Dettmer, Robert Stirling, Helen Chapel, Mary Lucas, Smita Patel, Claire Michele Farber, Isabelle Meyts, Arpan K. Banerjee, Scott Hackett, John R. Hurst, Klaus Warnatz, Benjamin Gathmann, Ulrich Baumann, for the Chest CT in Antibody Deficiency Group

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12 Citations (Scopus)

Abstract

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 20 years. 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.

Original languageEnglish
Pages (from-to)45-54
Number of pages10
JournalJournal of Clinical Immunology
Volume39
Issue number1
DOIs
Publication statusPublished - 15 Jan 2019
Externally publishedYes

Keywords

  • bronchial pathology
  • bronchiectasis
  • Chest CT
  • CVID
  • primary antibody deficiency

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