Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality

The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc). In this retrospective multi-center case-control study, we identified 36 SSc pat...

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Bibliographic Details
Published in:Seminars in arthritis and rheumatism 2019-08, Vol.49 (1), p.98-104
Main Authors: Champtiaux, N., Cottin, V., Chassagnon, G., Chaigne, B., Valeyre, D., Nunes, H., Hachulla, E., Launay, D., Crestani, B., Cazalets, C., Jego, P., Bussone, G., Bérezné, A., Guillevin, L., Revel, M.P., Cordier, J.F., Mouthon, L.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Case-Control Studies
Combined pulmonary fibrosis and emphysema
Female
Humans
Life Sciences
Lung - diagnostic imaging
Lung - physiopathology
Male
Middle Aged
Prognosis
Pulmonary Emphysema - complications
Pulmonary Emphysema - diagnostic imaging
Pulmonary Emphysema - physiopathology
Pulmonary Fibrosis - complications
Pulmonary Fibrosis - diagnostic imaging
Pulmonary Fibrosis - physiopathology
Pulmonary hypertension
Radiography, Thoracic
Respiratory Function Tests
Retrospective Studies
Scleroderma, Systemic - complications
Scleroderma, Systemic - diagnostic imaging
Scleroderma, Systemic - physiopathology
Survival
Systemic sclerosis
Tomography, X-Ray Computed
Young Adult
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Description
Summary:The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc). In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema. Rate of CPFE in SSc patients with CT scan was 3.6%, and 7.6% among SSc patients with ILD. CPFE-SSc patients were more likely to be male (75 % vs 18%, p 
ISSN:0049-0172
1532-866X
DOI:10.1016/j.semarthrit.2018.10.011