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

نویسندگان

  • Diane Bouvry
  • Luc Mouthon
  • Pierre-Yves Brillet
  • Marianne Kambouchner
  • Jean-Pierre Ducroix
  • Vincent Cottin
  • Julien Haroche
  • Jean-Francois Viallard
  • Romain Lazor
  • François Lebargy
  • Abdellatif Tazi
  • Benoît Wallaert
  • Amar Smail
  • Jean-Luc Pellegrin
  • Hilario Nunes
  • Zahir Amoura
  • Jean-François Cordier
  • Dominique Valeyre
  • Jean-Marc Naccache
چکیده

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.

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عنوان ژورنال:
  • The European respiratory journal

دوره 41 1  شماره 

صفحات  -

تاریخ انتشار 2013