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

doi:10.1016/S0221-0363(09)70077-0

Journal de Radiologie

Volume 90, Issue 1, Part 1, January 2009, Pages 43-51

https://doi.org/10.1016/S0221-0363(09)70077-0 Get rights and content

Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests.

Purpose.

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.

Section snippets

Population d’étude

Cette étude rétrospective multicentrique a été menée par le GERM « O » P (Groupe d’Étude et de Recherche sur les Maladies « Orphelines » Pulmonaires) et a reçu l’aval du comité local d’éthique. Il n’était pas nécessaire d’obtenir un consentement éclairé signé des patients. La base de données du GERM « O » P a été déclarée aux autorités compétentes (commission nationale informatique et liberté). La sélection des patients, les critères d’inclusion et d’exclusion, ainsi que les caractéristiques de

Résultats

Il existait une bonne concordance entre les deux lecteurs pour l’évaluation de la sévérité des scores de TDM-HR (la déviation standard des différences inter-observateurs était de 5,9, 7,2 et 6,9 pour les scores de fibrose, d’emphysème, et de verre dépoli, respectivement). Les différences dans la quantification de l’extension des lésions (10 % pour un niveau donné) étaient observées dans 5,5 %, 12 % et 8,8 % pour les scores de fibrose, d’emphysème, et de verre dépoli, respectivement.

Discussion

Le syndrome emphysème/fibrose peut réaliser des tableaux TDM-HR distincts, allant d’un emphysème généralisé sévère avec quelques signes discrets de fibrose, à des lésions de fibrose avec quelques rares bulles d’emphysème dans les sommets. Cependant la majorité des patients peuvent être classés en 3 présentations TDM-HR spécifiques (Transition progressive, Emphysème para-septal, Entités séparées) en fonction du type d’emphysème observé et de la distribution des lésions entre elles.

Dans notre

Conclusion

En conclusion, bien que posant des problèmes de diagnostic clinique et radiologique, le syndrome emphysème/fibrose a des présentations TDM-HR spécifiques avec différents types d’associations lésionnelles identifiables (Transition progressive, Emphysème para-septal, Entités séparées) qui peuvent être en rapport avec des mécanismes physiopathologiques différents. Notre étude souligne l’importance pour le radiologue de confronter les données TDM-HR aux données fonctionnelles dans les situations

Références (23)

Doherty MJ, Pearson MG, O’Grady EA, Pellegrini V, Calverley PM. Cryptogenic fibrosing alveolitis with preserved lung...
Hiwatari N, Shimura S, Takishima T. Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause....
Wiggins J, Strickland B, Turner-Warwick M. Combined cryptogenic fibrosing alveolitis and emphysema: the value of high...
Wells AU, Desai SR, Rubens MB, et al. Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease...
Suki B, Lutchen KR, Ingenito EP. On the progressive nature of emphysema: roles of proteases, inflammation, and...
Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA. Cigarette smoking: a risk factor for idiopathic pulmonary...
Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur...
Mura M, Zompatori M, Pacilli AM, et al. The presence of emphysema further impairs physiologic function in patients with...
Grubstein A, Bendayan D, Schactman I, et al. Concomitant upper-lobe bullous emphysema, lower-lobe interstitial fibrosis...
Gauldie J, Kolb M, Ask K, et al. Smad3 signaling involved in pulmonary fibrosis and emphysema. Proc Am Thorac Soc...

Marten K, Hansell DM. Imaging of macrophage-related lung diseases. Eur Radiol...

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

Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests.

Purpose.

Patients and methods.

Results.

Conclusion.

Objectifs.

Patients et méthodes.

Résultats.

Conclusion.

Section snippets

Population d’étude

Résultats

Discussion

Conclusion

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