[Diffuse interstitial pneumonia revealing Erdheim-Chester's disease]. / Pneumopathie interstitielle diffuse révélant une maladie d'Erdheim-Chester.
Rev Mal Respir
; 11(3): 304-7, 1994.
Article
en Fr
| MEDLINE
| ID: mdl-8041998
ABSTRACT
Erdheim-Chester's disease is a rare form of visceral xanthogranulomatosis. We report a case of a patient aged 50 presenting with a diffuse interstitial pneumonia which revealed Erdheim-Chester's disease with localisation in the bones, peri-aortic region and also with neurological involvement. The diffuse interstitial pneumonia which progressed chronically was characterised by a diffuse thickening of the septa with subpleural cysts and bilateral apical bullae with thickening of the pleura. Respiratory function tests showed a restrictive ventilatory defect with resting hypoxaemia which was aggravating by exercise. Broncho-alveolar lavage showed a lymphocytosis (26%) also with polymorpho neutrophils (11%). The pathological diagnosis was confirmed by transbronchial lung biopsy showing an excess of foamy histiocytes in the interstitium which are characteristic of the disease. The progress of this interstitial pneumonia is stabilised without immunosuppressive drugs.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades Óseas
/
Histiocitosis de Células no Langerhans
/
Enfermedades Pulmonares Intersticiales
/
Enfermedades del Sistema Nervioso
Límite:
Female
/
Humans
/
Middle aged
Idioma:
Fr
Revista:
Rev Mal Respir
Año:
1994
Tipo del documento:
Article