Combined pulmonary fibrosis and emphysema ...
Type de document :
Article dans une revue scientifique
PMID :
URL permanente :
Titre :
Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.
Auteur(s) :
Champtiaux, Nicolas [Auteur]
Cottin, Vincent [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Chassagnon, G [Auteur]
Chaigne, Benjamin [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Valeyre, Dominique [Auteur]
Université Sorbonne Paris Nord
Nunes, H [Auteur]
Université Sorbonne Paris Nord
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Launay, David [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Crestani, Bruno [Auteur]
Université Paris Diderot - Paris 7 [UPD7]
Cazalets, Claire [Auteur]
Jego, Patrick [Auteur]
Bussone, G [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Berezne, Alice [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Guillevin, Loic [Auteur]
Université Paris Cité [UPCité]
Revel M, P [Auteur]
Mouthon, Luc [Auteur]
Cottin, Vincent [Auteur]
Université Claude Bernard Lyon 1 [UCBL]
Chassagnon, G [Auteur]
Chaigne, Benjamin [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Valeyre, Dominique [Auteur]
Université Sorbonne Paris Nord
Nunes, H [Auteur]
Université Sorbonne Paris Nord
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Launay, David [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Crestani, Bruno [Auteur]
Université Paris Diderot - Paris 7 [UPD7]
Cazalets, Claire [Auteur]
Jego, Patrick [Auteur]
Bussone, G [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Berezne, Alice [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Guillevin, Loic [Auteur]
Université Paris Cité [UPCité]
Revel M, P [Auteur]
Mouthon, Luc [Auteur]
Titre de la revue :
Seminars in Arthritis and Rheumatism
Nom court de la revue :
Semin. Arthritis Rheum.
Numéro :
49
Pagination :
98-104
Date de publication :
2019-08
ISSN :
1532-866X
Discipline(s) HAL :
Sciences du Vivant [q-bio]
Résumé en anglais : [en]
BACKGROUND: 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 ...
Lire la suite >BACKGROUND: 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). METHODS: 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. RESULTS: 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 < 0.0001), smokers (83 % vs 33%, p < 0.0001), and to have limited cutaneous SSc (53 % vs 24% p < 0.01) than ILD-SSc controls. No specific autoantibody was significantly associated with CPFE. At diagnosis, CPFE-SSc patients had a greater decrease in carbon monoxide diffusing capacity (DLCO 39 +/- 13 % vs 51 +/- 12% of predicted value, p < 0.0001) when compared to SSc-ILD controls, whereas lung volumes (total lung capacity and forced vital capacity) were similar. During follow-up, CPFE-SSc patients more frequently developed precapillary pulmonary hypertension (PH) (44 % vs 11%, p < 10(-4)), experienced more frequent unscheduled hospitalizations (50 % vs 25%, p < 0.01), and had decreased survival (p < 0.02 by Kaplan-Meier survival analysis) as compared to ILD-SSc controls. CONCLUSIONS: The CPFE syndrome is a distinct pulmonary manifestation in SSc, with higher morbidity and mortality. Early diagnosis of CPFE by chest CT in SSc patients (especially smokers) may result in earlier smoking cessation, screening for PH, and appropriate management.Lire moins >
Lire la suite >BACKGROUND: 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). METHODS: 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. RESULTS: 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 < 0.0001), smokers (83 % vs 33%, p < 0.0001), and to have limited cutaneous SSc (53 % vs 24% p < 0.01) than ILD-SSc controls. No specific autoantibody was significantly associated with CPFE. At diagnosis, CPFE-SSc patients had a greater decrease in carbon monoxide diffusing capacity (DLCO 39 +/- 13 % vs 51 +/- 12% of predicted value, p < 0.0001) when compared to SSc-ILD controls, whereas lung volumes (total lung capacity and forced vital capacity) were similar. During follow-up, CPFE-SSc patients more frequently developed precapillary pulmonary hypertension (PH) (44 % vs 11%, p < 10(-4)), experienced more frequent unscheduled hospitalizations (50 % vs 25%, p < 0.01), and had decreased survival (p < 0.02 by Kaplan-Meier survival analysis) as compared to ILD-SSc controls. CONCLUSIONS: The CPFE syndrome is a distinct pulmonary manifestation in SSc, with higher morbidity and mortality. Early diagnosis of CPFE by chest CT in SSc patients (especially smokers) may result in earlier smoking cessation, screening for PH, and appropriate management.Lire moins >
Langue :
Anglais
Audience :
Internationale
Vulgarisation :
Non
Établissement(s) :
Inserm
Université de Lille
CHU Lille
Université de Lille
CHU Lille
Équipe(s) de recherche :
Immunity, inflammation and fibrsis in auto and allo-reactivity
Date de dépôt :
2019-03-01T14:17:32Z
2023-12-13T16:29:25Z
2023-12-13T16:29:25Z
Fichiers
- document
- Accès libre
- Accéder au document