ERJ
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Permissions
Right arrowRequest Permissions
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Google Scholar
Right arrow Articles by Khaldi, H.
Right arrow Articles by Crestani, B.
PubMed
Right arrow PubMed Citation
Right arrow Articles by Khaldi, H.
Right arrow Articles by Crestani, B.
Eur Respir J 2009; 33:1498-1502
Copyright ©ERS Journals Ltd 2009

Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease

H. Khaldi1,2, S. Marchand-Adam1, C. Kannengiesser3,4, A. Fabre5, M. P. Debray6, C. Danel3, M-A. Gougerot-Pocidalo4,7,8, J. El Benna8, M. Humbert9, M. Aubier1 and B. Crestani1

1 APHP, Service de Pneumologie, 3 Service de Génétique, 5 Service d'Anatomie Pathologique, 6 Service de Radiologie, 7 Service d'Immunologie et d'Hématologie, Hôpital Bichat, 4 CIB Phenogen Paris, 8 INSERM U773, Paris, 9 APHP, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Beclère, Clamart, France. 2 Service de pneumologie, Hôpital Abderrahmen Mami, Ariana, Tunisie.

CORRESPONDENCE: B. Crestani, Service de Pneumologie Hôpital Bichat, 16 rue Henri Huchard, 75877, Paris cedex 18, France. E-mail: bruno.crestani{at}bch.aphp.fr

Keywords: Chronic granulomatous disease, lung fibrosis, pigmented macrophages, pulmonary hypertension, pulmonary veno-occlusive disease

Received: October 19, 2007
Accepted January 13, 2009

The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension.

Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease.

Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level.

In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2009 by the European Respiratory Society.