Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease.

Détails

ID Serval
serval:BIB_4CAF8401BB7E
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease.
Périodique
Arthritis and Rheumatism
Auteur⸱e⸱s
Cottin V., Nunes H., Mouthon L., Gamondes D., Lazor R., Hachulla E., Revel D., Valeyre D., Cordier J.F.
Collaborateur⸱rice⸱s
Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires
Contributeur⸱rice⸱s
Bérezné A., Bardin T., Hirschi S., Hyvernat P., Kessler R., Khouatra C., Lioté H., Marchand E., Mornex JF., Stern M., Tazi A.
ISSN
1529-0131 (Electronic)
ISSN-L
0004-3591
Statut éditorial
Publié
Date de publication
2011
Volume
63
Numéro
1
Pages
295-304
Langue
anglais
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
Résumé
OBJECTIVE: Connective tissue diseases (CTDs) are associated with several interstitial lung diseases. The aim of this study was to describe the recently individualized syndrome of combined pulmonary fibrosis and emphysema (CPFE) in a population of patients with CTD.
METHODS: In this multicenter study, we retrospectively investigated data from patients with CTD who also have CPFE. The demographic characteristics of the patients, the results of pulmonary function testing, high-resolution computed tomography, lung biopsy, and treatment, and the outcomes of the patients were analyzed.
RESULTS: Data from 34 patients with CTD who were followed up for a mean±SD duration of 8.3±7.0 years were analyzed. Eighteen of the patients had rheumatoid arthritis (RA), 10 had systemic sclerosis (SSc), 4 had mixed or overlap CTD, and 2 had other CTDs. The mean±SD age of the patients was 57±11 years, 23 were men, and 30 were current or former smokers. High-resolution computed tomography revealed emphysema of the upper lung zones and pulmonary fibrosis of the lower zones in all patients, and all patients exhibited dyspnea during exercise. Moderately impaired pulmonary function test results and markedly reduced carbon monoxide transfer capacity were observed. Five patients with SSc exhibited pulmonary hypertension. Four patients died during followup. Patients with CTD and CPFE were significantly younger than an historical control group of patients with idiopathic CPFE and more frequently were female. In addition, patients with CTD and CPFE had higher lung volumes, lower diffusion capacity, higher pulmonary pressures, and more frequently were male than those with CTD and lung fibrosis without emphysema.
CONCLUSION: CPFE warrants inclusion as a novel, distinct pulmonary manifestation within the spectrum of CTD-associated lung diseases in smokers or former smokers, especially in patients with RA or SSc.
Mots-clé
Aged, Connective Tissue Diseases/complications, Connective Tissue Diseases/pathology, Female, Humans, Hypertension, Pulmonary/complications, Hypertension, Pulmonary/pathology, Lung/pathology, Lung/physiopathology, Male, Middle Aged, Pulmonary Emphysema/complications, Pulmonary Emphysema/pathology, Pulmonary Fibrosis/complications, Pulmonary Fibrosis/pathology, Respiratory Function Tests, Retrospective Studies, Smoking, Syndrome
Pubmed
Web of science
Création de la notice
15/02/2011 13:37
Dernière modification de la notice
20/08/2019 14:01
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