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

. 2019 Jan ; 39 (1) : 45-54. [epub] 20181213

Language English Country Netherlands Media print-electronic

Document type Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't

Links

PubMed 30547383
DOI 10.1007/s10875-018-0577-9
PII: 10.1007/s10875-018-0577-9
Knihovny.cz E-resources

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.

Allergy Immunology and Respiratory Medicine The Alfred Hospital Melbourne Australia

Centre for Chronic Immunodeficiency University Medical Center of Freiburg Freiburg Germany

Clinical Immunology Royal Free Hospital London UK

Department of Clinical Immunology and Allergy Faculty of Medicine Masaryk University St Anne's University Hospital Brno Czech Republic

Department of Molecular Medicine Sapienza University of Rome Rome Italy

Department of Radiology Erasmus MC Sophia Children's Hospital Rotterdam The Netherlands

Department of Radiology Faculty of Medicine Masaryk University St Anne's University Hospital Brno Czech Republic

Department of Radiology University of Brescia Brescia Italy

Department of Respiratory Medicine Royal Brompton Hospital London UK

Diagnostic Radiology Hanover Medical School Hanover Germany

Dutch Cancer Institute Antoni van Leeuwenhoek Hospital The Hague The Netherlands

ESID Registry Working Party University Hospital Freiburg Freiburg Germany

Immunology Addenbrooke's Hospital Cambridge UK

Immunology and Internal Medicine Erasmus MC Rotterdam The Netherlands

Immunology Cliniques Universitaires de Bruxelles Hôpital Erasme Brussels Belgium

Immunology Papworth Hospital Cambridge UK

Immunology Section Department of Medicine Imperial College London London UK

Immunology University of Naples Federico 2 Naples Italy

Institute of Nuclear Medicine University College London London UK

Jeroen Bosch Academy Jeroen Bosch Hospital 's Hertogenbosch The Netherlands

Paediatric Immunology and Pulmonology University Hospitals Leuven Belgium

Paediatric Immunology Department Heartlands Hospital Birmingham Birmingham UK

Paediatric Immunology Erasmus MC Sophia Children's Hospital Rotterdam The Netherlands

Paediatric Immunology Great Ormond Street Hospital London UK

Paediatric Immunology Unit Department of Paediatric Pulmonology Allergology and Neonatology Hanover Medical School Carl Neuberg Str 1 30625 Hannover Germany

Paediatric University Hospital Cairo Egypt

Paediatrics Juliana Children's Hospital Haga Teaching Hospital The Hague The Netherlands

Pediatrics Clinic and Institute for Molecular Medicine A Nocivelli Department of Clinical and Experimental Sciences University of Brescia and ASST Spedali Civili of Brescia Brescia Italy

Pediatrics Clinic ASST Spedali Civili Brescia Italy

Primary Immunodeficiencies Unit Pediatrics Hospital 12 Octubre Madrid Spain

Primary Immunodeficiency Unit Nuffield Department of Medicine University of Oxford Oxford UK

Radiology Addenbrooke's Hospital Cambridge UK

Radiology Heartlands Hospital Birmingham UK

Radiology IBB CNR University of Naples Federico 2 Naples Italy

Radiology Papworth Hospital Cambridge UK

Radiology Royal Free Hospital London UK

Radiology Università degli Studi di Roma La Sapienza Rome Italy

RECETOX Faculty of Science Masaryk University Brno Czech Republic

Tranzo Tilburg University Tilburg The Netherlands

UCL Respiratory Medicine University College London London UK

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