N Champtiaux1, V Cottin2, G Chassagnon3, B Chaigne1, D Valeyre4, H Nunes4, E Hachulla5, D Launay5, B Crestani6, C Cazalets7, P Jego7, G Bussone1, A Bérezné1, L Guillevin1, M P Revel3, J F Cordier2, L Mouthon8. 1. Department of Internal Medicine, Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, DHU Authors (Autoimmune and Hormonal Diseases), Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France. 2. Service de Pneumologie, Centre National de Référence des maladies pulmonaire rares, Hospices Civils de Lyon, Hôpital Louis Pradel, Groupe d'Etudes et de Recherche sur les Maladies « Orphelines » Pulmonaires (GERM«O»P), Université Claude Bernard Lyon 1, UMR754, Lyon, France. 3. Service de Radiologie, Hôpital Cochin, France. 4. Service de Pneumologie, APHP, hôpital Avicenne, Université Paris Nord, 93000 Bobigny, France. 5. Université de Lille, Inserm, CHU Lille, U995 - LIRIC - Lille Inflammation Research International Center, Service de Médecine Interne, Hôpital Claude Huriez, Centre de Référence pour la Sclérodermie Systémique, FHU IMMInENT, F-59000 Lille, France. 6. Service de Pneumologie A, Hôpital Bichat, DHU FIRE, Université Paris Diderot, Paris, France. 7. Service de médecine interne, CHU de Rennes, 2, rue Henri-Le-Guilloux, 35000 Rennes, France. 8. Department of Internal Medicine, Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies Systémiques Autoimmunes Rares d'Ile de France, DHU Authors (Autoimmune and Hormonal Diseases), Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), 27, rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France. Electronic address: luc.mouthon@aphp.fr.
Abstract
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.
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.
Authors: Sangmee Sharon Bae; Lila Pourzand; Grace Hyun Kim; Bianca E Villegas; Andrea Oh; Daniel E Furst; Jonathan Goldin; Donald P Tashkin Journal: J Scleroderma Relat Disord Date: 2021-10-23
Authors: Nicholas Landini; Martina Orlandi; Cosimo Bruni; Edoardo Carlesi; Cosimo Nardi; Linda Calistri; Giovanni Morana; Sara Tomassetti; Stefano Colagrande; Marco Matucci-Cerinic Journal: Front Med (Lausanne) Date: 2022-01-27
Authors: Anna Stainer; Alex Rice; Anand Devaraj; Joseph Luke Barnett; Jacqueline Donovan; Maria Kokosi; Andrew Gordon Nicholson; Tom Cairns; Athol Umfrey Wells; Elisabetta Augusta Renzoni Journal: BMC Pulm Med Date: 2019-10-24 Impact factor: 3.320