Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis.
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.
Assistance Publique – Hôpitaux de Paris, Service de Pneumologie, and Université Paris 13, PRES Sorbonne-Paris-Cité, EA 2363, Hôpital Universitaire Avicenne, Bobigny, France.
Diane
D
Bouvry
Luc
L
Mouthon
Pierre-Yves
PY
Brillet
Marianne
M
Kambouchner
Jean-Pierre
JP
Ducroix
Vincent
V
Cottin
Julien
J
Haroche
Jean-Francois
JF
Viallard
Romain
R
Lazor
François
F
Lebargy
Abdellatif
A
Tazi
Benoît
B
Wallaert
Amar
A
Smail
Jean-Luc
JL
Pellegrin
Hilario
H
Nunes
Zahir
Z
Amoura
Jean-François
JF
Cordier
Dominique
D
Valeyre
Jean-Marc
JM
Naccache
D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006099:Granuloma / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D017565:Sarcoidosis, Pulmonary / Q000175:diagnosis*; D055815:Young Adult