Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure

International audienceTwo case histories are described of pleural and anterior mediastinal fibrosis presenting as a continuous fibrotic process with thick parietal pleural plaques extending from one pleura to the contralateral pleura through the retrosternal area, and with retroperitoneal fibrosis. Followup over 4 years in one case demonstrated rapid progression of disease, with pleural fibrosis preceding retrosternal and retroperitoneal fibrosis. Histopathological analysis in both cases showed non-tumoral fibrosis with broad fibrous bundles surrounding fibroblasts (and lymphocytes in one case). Possible causes such as infections and exposure to ergot derivatives were excluded. Both patients had been slightly or moderately exposed to asbestos. These cases represent an unusual new presentation of pleural and retrosternal fibrosis extending beyond the anatomical structures and associated with retroperitoneal fibrosis

Syndrome emphysème des sommets et fibrose pulmonaire des bases combinés (syndrome emphysème/fibrose) : aspects tomodensitométriques et fonctionnels

National audiencePurpose. To describe the high resolution CT (HRCT) imaging and functional features of the emphysema/fibrosis syndrome. Patients and methods. A total of 61 patients were included based on HRCT. We have quantified the extent of fibrosis and emphysema lesions and a combined score was calculated. The scores were correlated to pulmonary function test parameters and specific HRCT features were described. Results. The emphysema and fibrosis scores correlated with functional parameters of obstruction and restriction respectively. The combined score correlated with the reduction in DLCO and degree of pulmonary hypertension. Three HRCT patterns were identified : progressive transition (n = 23, 38%) with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing ; paraseptal emphysema (n = 13, 21%) with predominent subpleural bullae of enlarging size at the bases ; separate processes (n = 14, 23%) with independent areas of fibrosis and emphysema. Eleven patients (18%) could not be classified. The HRCT imaging features changed based on TLC (p = 0.04) and FEV1/FVC (p = 0.01). Conclusion. The emphysema/fibrosis syndrome may be associated with different patterns on HRCT corresponding to specific profiles on pulmonary function tests.Objectifs. Décrire les aspects tomodensitométriques en haute résolution (TDM-HR) et fonctionnels associés au syndrome emphysème/fibrose. Patients et méthodes. Soixante et un patients ont été inclus sur la base de la TDM-HR. Nous avons quantifié l’extension des lésions de fibrose et d’emphysème et nous avons calculé un score combiné. Ces scores ont été corrélés aux paramètres fonctionnels puis les tableaux TDM-HR spécifiques de ce syndrome ont été décrits. Résultats. Les scores d’emphysème et de fibrose étaient corrélés avec les paramètres fonctionnels d’obstruction et de restriction, respectivement. Le score combiné était corrélé à la réduction de la DLCO et au niveau d’hypertension pulmonaire. Nous avons identifié trois tableaux TDM-HR : Transition progressive (n = 23, 38 %) consistant en l’association d’un emphysème diffus (centro-lobulaire et/ou bulleux) et la présence d’une zone de transition entre les bulles et le rayon de miel ; Emphysème para-septal (n = 13, 21 %) consistant en des bulles sous pleurales prédominantes augmentant de taille dans les bases pulmonaires ; Entités séparées (n = 14, 23 %) où la fibrose et l’emphysème n’avaient pas de relation topographique. Onze patients (18 %) ne pouvaient être classés. Les présentations TDM-HR différaient en fonction de la CPT (p = 0,04) et du rapport VEMS/CVF (p = 0,01). Conclusion. Le syndrome emphysème/fibrose peut réaliser des tableaux TDM-HR distincts qui sont associés à des profils fonctionnels spécifiques

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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

Combined pulmonary fibrosis and emphysema : a distinct underrecognised entity

International audienceThe syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described.The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean±sd): total lung capacity 88%±17, forced vital capacity (FVC) 88%±18, forced expiratory volume in one second (FEV1) 80%±21 (% predicted), FEV1/FVC 69%±13, carbon monoxide diffusion capacity of the lung 37%±16 (% predicted), carbon monoxide transfer coefficient 46%±19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1±2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival