Abstract
To investigate to what extent interstitial lung disease in common variable immunodeficiency associated granulomatous disease can be found similar to pulmonary sarcoidosis
Twenty patients with common variable immunodeficiency associated with granulomatous disease were included in a retrospective study conducted by the Groupe Sarcoïdose Francophone. Medical records were centralized. Patients were compared with 60 controls with sarcoidosis.
Clinical examination showed more frequent crackles in patients than in controls (45% versus 1.7%, p<0.001). On thoracic computed tomography scans, nodules, often multiple, with smooth margin, air-bronchogram and halo sign, were more frequent in patients (80% versus 42%, p=0.004) as well as bronchiectasis (65% versus 23%, p<0.001). The micronodule's distribution was perilymphatic in 100% of controls while in 42% of patients (p<0.001). Bronchoalveolar lavage analysis showed lower T-lymphocyte CD4/CD8 ratio in patients than in controls (1.6+/−1.1 versus 5.3+/−4, 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 (30% versus 0) and resulted from common variable immunodeficiency complications.
Interstitial lung disease in common variable immunodeficiency associated with granulomatous disease presents a specific picture and evolution markedly different from that of sarcoidosis.
- ERS
