Imaging of bronchial pathology in antibody deficiency: Data from the European Chest CT Group

Chest CT in Antibody Deficiency Group

Research output: Contribution to journalArticleScientificpeer-review

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
JournalJournal of Clinical Immunology
Volume39
Issue number1
DOIs
Publication statusPublished - 2019

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Pathology
Common Variable Immunodeficiency
Immunologic Deficiency Syndromes
Multicenter Studies
Delayed Diagnosis
Cough

Keywords

  • COMMON VARIABLE IMMUNODEFICIENCY
  • COMPUTED-TOMOGRAPHY
  • CVID
  • CYSTIC FIBROSIS BRONCHIECTASIS
  • Chest CT
  • DISEASES
  • LARGE COHORT
  • LUNG-FUNCTION
  • MANAGEMENT
  • PULMONARY-FUNCTION
  • SCAN
  • X-LINKED AGAMMAGLOBULINEMIA
  • bronchial pathology
  • bronchiectasis
  • primary antibody deficiency

Cite this

Chest CT in Antibody Deficiency Group. / Imaging of bronchial pathology in antibody deficiency : Data from the European Chest CT Group. In: Journal of Clinical Immunology. 2019 ; Vol. 39, No. 1. pp. 45-54.
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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.",
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author = "{Chest CT in Antibody Deficiency Group} and K. Sch{\"u}tz and D. Alecsandru and B. Grimbacher and J. Haddock and A. Bruining and G. Driessen and {de Vries}, E. and {van Hagen}, P.M. and I. Hartmann and F. Fraioli and C. Milito and M. Mitrevski and I. Quinti and G. Serra and P. Kelleher and M. Loebinger and J. Litzman and V. Postranecka and V. Thon and J. Babar and A.M. Condliffe and A. Exley and D. Kumararatne and N. Screaton and A. Jones and M.P. Bondioni and V. Lougaris and A. Plebani and A. Soresina and C. Sirignano and G. Spadaro and Nermeen Galal and L.I. Gonzalez-Granado and S. Dettmer and R. Stirling and H. Chapel and M. Lucas and S. Patel and C. Farber and I. Meyts and A.K. Banerjee and S. Hackett and J.R. Hurst and K. Warnatz and B. Gathmann and U. Baumann",
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Imaging of bronchial pathology in antibody deficiency : Data from the European Chest CT Group. / Chest CT in Antibody Deficiency Group.

In: Journal of Clinical Immunology, Vol. 39, No. 1, 2019, p. 45-54.

Research output: Contribution to journalArticleScientificpeer-review

TY - JOUR

T1 - Imaging of bronchial pathology in antibody deficiency

T2 - Data from the European Chest CT Group

AU - Chest CT in Antibody Deficiency Group

AU - Schütz, K.

AU - Alecsandru, D.

AU - Grimbacher, B.

AU - Haddock, J.

AU - Bruining, A.

AU - Driessen, G.

AU - de Vries, E.

AU - van Hagen, P.M.

AU - Hartmann, I.

AU - Fraioli, F.

AU - Milito, C.

AU - Mitrevski, M.

AU - Quinti, I.

AU - Serra, G.

AU - Kelleher, P.

AU - Loebinger, M.

AU - Litzman, J.

AU - Postranecka, V.

AU - Thon, V.

AU - Babar, J.

AU - Condliffe, A.M.

AU - Exley, A.

AU - Kumararatne, D.

AU - Screaton, N.

AU - Jones, A.

AU - Bondioni, M.P.

AU - Lougaris, V.

AU - Plebani, A.

AU - Soresina, A.

AU - Sirignano, C.

AU - Spadaro, G.

AU - Galal, Nermeen

AU - Gonzalez-Granado, L.I.

AU - Dettmer, S.

AU - Stirling, R.

AU - Chapel, H.

AU - Lucas, M.

AU - Patel, S.

AU - Farber, C.

AU - Meyts, I.

AU - Banerjee, A.K.

AU - Hackett, S.

AU - Hurst, J.R.

AU - Warnatz, K.

AU - Gathmann, B.

AU - Baumann, U.

PY - 2019

Y1 - 2019

N2 - 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.

AB - 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.

KW - COMMON VARIABLE IMMUNODEFICIENCY

KW - COMPUTED-TOMOGRAPHY

KW - CVID

KW - CYSTIC FIBROSIS BRONCHIECTASIS

KW - Chest CT

KW - DISEASES

KW - LARGE COHORT

KW - LUNG-FUNCTION

KW - MANAGEMENT

KW - PULMONARY-FUNCTION

KW - SCAN

KW - X-LINKED AGAMMAGLOBULINEMIA

KW - bronchial pathology

KW - bronchiectasis

KW - primary antibody deficiency

U2 - 10.1007/s10875-018-0577-9

DO - 10.1007/s10875-018-0577-9

M3 - Article

VL - 39

SP - 45

EP - 54

JO - Journal of Clinical Immunology

JF - Journal of Clinical Immunology

SN - 0271-9142

IS - 1

ER -