Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis.

Détails

ID Serval
serval:BIB_7E741BAD1D1D
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis.
Périodique
European Respiratory Journa
Auteur(s)
Bouvry D., Mouthon L., Brillet P.Y., Kambouchner M., Ducroix J.P., Cottin V., Haroche J., Viallard J.F., Lazor R., Lebargy F., Tazi A., Wallaert B., Smail A., Pellegrin J.L., Nunes H., Amoura Z., Cordier J.F., Valeyre D., Naccache J.M.
Collaborateur(s)
the Groupe Sarcoïdose Francophone
ISSN
1399-3003 (Electronic)
ISSN-L
0903-1936
Statut éditorial
Publié
Date de publication
2013
Volume
41
Numéro
1
Pages
115-122
Langue
anglais
Notes
Publication types: JOURNAL ARTICLE
Résumé
The aim of the present study was to investigate to what extent interstitial lung disease (ILD) in common variable immunodeficiency disorder (CVID)-associated granulomatous disease (GD) is similar to pulmonary sarcoidosis 20 patients with CVID/GD were included in a retrospective study conducted by the Groupe Sarcoïdose Francophone. Medical records were centralised. Patients were compared with 60 controls with sarcoidosis. Clinical examination showed more frequent crackles in patients than controls (45% versus 1.7%, respectively; p<0.001). On thoracic computed tomography scans, nodules (often multiple and with smooth margins), air bronchograms and halo signs were more frequent in patients than controls (80% versus 42%, respectively; p=0.004) as well as bronchiectasis (65% versus 23%, respectively; p<0.001). The micronodule distribution was perilymphatic in 100% of controls and in 42% of patients (p<0.001). Bronchoalveolar lavage analysis showed lower T-cell CD4/CD8 ratios in patients than in controls (mean±sd 1.6±1.1 versus 5.3±4, respectively; p<0.01). On pathological analysis, nodules and consolidations corresponded to granulomatous lesions with or without lymphocytic disorders in most cases. Mortality was higher in patients than controls (30% versus 0%, respectively) and resulted from common variable immunodeficiency complications. ILD in CVID/GD presents a specific clinical picture and evolution that are markedly different from those of sarcoidosis.
Pubmed
Web of science
Open Access
Oui
Création de la notice
07/02/2013 18:59
Dernière modification de la notice
08/05/2019 21:00
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