Document Detail

Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease.
MedLine Citation:
PMID:  20936629     Owner:  NLM     Status:  MEDLINE    
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.
Vincent Cottin; Hilario Nunes; Luc Mouthon; Delphine Gamondes; Romain Lazor; Eric Hachulla; Didier Revel; Dominique Valeyre; Jean-François Cordier;
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Publication Detail:
Type:  Journal Article; Multicenter Study    
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  63     ISSN:  1529-0131     ISO Abbreviation:  Arthritis Rheum.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-01-04     Completed Date:  2011-01-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  United States    
Other Details:
Languages:  eng     Pagination:  295-304     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2011 by the American College of Rheumatology.
Hospices Civils de Lyon, Hôpital Louis Pradel, Université de Lyon, UMR754, and IFR128, Lyon, France.
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MeSH Terms
Connective Tissue Diseases / complications,  pathology*,  physiopathology,  radiography
Hypertension, Pulmonary / complications,  pathology,  physiopathology,  radiography
Lung / pathology*,  physiopathology,  radiography
Middle Aged
Pulmonary Emphysema / complications,  pathology*,  physiopathology,  radiography
Pulmonary Fibrosis / complications,  pathology*,  physiopathology,  radiography
Respiratory Function Tests
Retrospective Studies
A Bérezné / ; T Bardin / ; S Hirschi / ; P Hyvernat / ; R Kessler / ; C Khouatra / ; H Lioté / ; E Marchand / ; J F Mornex / ; M Stern / ; A Tazi /

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