Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis
Objectives This multicentre study aimed to investigate the overall mortality of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) and to compare CPFE-SSc characteristics with those of other SSc subtypes (with interstitial lung disease—ILD, emphysema or neither).Methods Che...
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BMJ Publishing Group
2019-05-01
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| author | Carlo Alberto Scirè Alarico Ariani Mario Silva Elena Bravi Simone Parisi Marta Saracco Fabio De Gennaro Cristian Caimmi Francesco Girelli Maria De Santis Alessandro Volpe Federica Lumetti Vanessa Hax Markus Bredemeier Veronica Alfieri Daniele Santilli Flavio Cesare Bodini Gianluca Lucchini Flavio Mozzani Valeria Seletti Emanuele Bacchini Eugenio Arrigoni Dilia Giuggioli Rafael Chakr Luca Idolazzi Giuseppina Bertorelli Davide Imberti Emanuele Michieletti Giuseppe Paolazzi Enrico Fusaro Alfredo Antonio Chetta Nicola Sverzellati |
| author_facet | Carlo Alberto Scirè Alarico Ariani Mario Silva Elena Bravi Simone Parisi Marta Saracco Fabio De Gennaro Cristian Caimmi Francesco Girelli Maria De Santis Alessandro Volpe Federica Lumetti Vanessa Hax Markus Bredemeier Veronica Alfieri Daniele Santilli Flavio Cesare Bodini Gianluca Lucchini Flavio Mozzani Valeria Seletti Emanuele Bacchini Eugenio Arrigoni Dilia Giuggioli Rafael Chakr Luca Idolazzi Giuseppina Bertorelli Davide Imberti Emanuele Michieletti Giuseppe Paolazzi Enrico Fusaro Alfredo Antonio Chetta Nicola Sverzellati |
| author_sort | Carlo Alberto Scirè |
| collection | DOAJ |
| description | Objectives This multicentre study aimed to investigate the overall mortality of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) and to compare CPFE-SSc characteristics with those of other SSc subtypes (with interstitial lung disease—ILD, emphysema or neither).Methods Chest CTs, anamnestic data, immunological profile and pulmonary function tests of patients with SSc were retrospectively collected. Each chest CT underwent a semiquantitative assessment blindly performed by three radiologists. Patients were clustered in four groups: SSc-CPFE, SSc-ILD, SSc-emphysema and other-SSc (without ILD nor emphysema). The overall mortality of these groups was calculated by Kaplan-Meier method and compared with the stratified log-rank test; Kruskal-Wallis test, t-Student test and χ² test assessed the differences between groups. P<0.05 was considered statistically significant.Results We enrolled 470 patients (1959 patient-year); 15.5 % (73/470) died during the follow-up. Compared with the SSc-ILD and other-SSc, in SSc-CPFE there was a higher prevalence of males, lower anticentromere antibodies prevalence and a more reduced pulmonary function (p<0.05). The Kaplan-Meier survival analysis demonstrates a significantly worse survival in patients with SSc-CPFE (HR vs SSc-ILD, vs SSc-emphysema and vs other-SSc, respectively 1.6 (CI 0.5 to 5.2), 1.6 (CI 0.7 to 3.8) and 2.8 (CI 1.2 to 6.6).Conclusions CPFE increases the mortality risk in SSc along with a highly impaired lung function. These findings strengthen the importance to take into account emphysema in patients with SSc with ILD. |
| format | Article |
| id | doaj-art-16fdb7f0980a4e0f934f2954827b9377 |
| institution | OA Journals |
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| language | English |
| publishDate | 2019-05-01 |
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| spelling | doaj-art-16fdb7f0980a4e0f934f2954827b93772025-08-20T02:26:20ZengBMJ Publishing GroupRMD Open2056-59332019-05-015110.1136/rmdopen-2018-000820Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosisCarlo Alberto Scirè0Alarico Ariani1Mario Silva2Elena Bravi3Simone Parisi4Marta Saracco5Fabio De Gennaro6Cristian Caimmi7Francesco Girelli8Maria De Santis9Alessandro Volpe10Federica Lumetti11Vanessa Hax12Markus Bredemeier13Veronica Alfieri14Daniele Santilli15Flavio Cesare Bodini16Gianluca Lucchini17Flavio Mozzani18Valeria Seletti19Emanuele Bacchini20Eugenio Arrigoni21Dilia Giuggioli22Rafael Chakr23Luca Idolazzi24Giuseppina Bertorelli25Davide Imberti26Emanuele Michieletti27Giuseppe Paolazzi28Enrico Fusaro29Alfredo Antonio Chetta30Nicola Sverzellati31Epidemiology Unit, Italian Society for Rheumatology (SIR), Milan, Italy1 Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy2 Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy3 Department of Medicine, Internal Medicine and Rheumatology Unit, Ospedale G. Da Saliceto, Piacenza, Italy4 Rheumatology Department, Azienda Ospedaliera Universitaria Città della Salute e della Scienza di Torino, Turin, Italy5 Rheumatology Unit, Ospedale Mauriziano – Umberto I di Torino, Torino, Italy6 Rheumatology Unit, Azienda Ospedaliera “Istituti Ospitalieri” di Cremona, Cremona, Italy7 Department of Medicine, Rheumatology Unit, University of Verona, Azienda Ospedaliera di Verona, Verona, Italy8 Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni – L Pierantoni, Forlì, ItalyDepartment of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy10 Rheumatology Unit, Ospedale di Trento, Trento, Italy11 Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy12 Division of Rheumatology, Universidade Federal do Rio Grande do Sul, Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil13 Rheumatology Service, Hospital Nossa Senhora da Conceição – Grupo Hospitalar Conceição, Porto Alegre, Brazil14 Department of Medicine and Surgery (DiMeC), Respiratory Disease Unit, University of Parma, Parma, Italy1 Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy15 Radiology Unit, Ospedale G. Da Saliceto, Piacenza, Italy1 Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy1 Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy2 Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy2 Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, Italy3 Department of Medicine, Internal Medicine and Rheumatology Unit, Ospedale G. Da Saliceto, Piacenza, Italy11 Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria, Policlinico di Modena, Modena, Italy16 Rheumatology, Hospital de Clínicas de Porto Alegre, Porto Alegre, BrazilSection of Rheumatology, Università degli Studi di Verona, Verona, Italy14 Department of Medicine and Surgery (DiMeC), Respiratory Disease Unit, University of Parma, Parma, Italy3 Department of Medicine, Internal Medicine and Rheumatology Unit, Ospedale G. Da Saliceto, Piacenza, Italy15 Radiology Unit, Ospedale G. Da Saliceto, Piacenza, Italy10 Rheumatology Unit, Ospedale di Trento, Trento, Italy4 Rheumatology Department, Azienda Ospedaliera Universitaria Città della Salute e della Scienza di Torino, Turin, Italy14 Department of Medicine and Surgery (DiMeC), Respiratory Disease Unit, University of Parma, Parma, Italy2 Department of Medicine and Surgery (DiMeC), Unit of Surgical Sciences, Section of Radiology, University of Parma, Parma, ItalyObjectives This multicentre study aimed to investigate the overall mortality of combined pulmonary fibrosis and emphysema (CPFE) in systemic sclerosis (SSc) and to compare CPFE-SSc characteristics with those of other SSc subtypes (with interstitial lung disease—ILD, emphysema or neither).Methods Chest CTs, anamnestic data, immunological profile and pulmonary function tests of patients with SSc were retrospectively collected. Each chest CT underwent a semiquantitative assessment blindly performed by three radiologists. Patients were clustered in four groups: SSc-CPFE, SSc-ILD, SSc-emphysema and other-SSc (without ILD nor emphysema). The overall mortality of these groups was calculated by Kaplan-Meier method and compared with the stratified log-rank test; Kruskal-Wallis test, t-Student test and χ² test assessed the differences between groups. P<0.05 was considered statistically significant.Results We enrolled 470 patients (1959 patient-year); 15.5 % (73/470) died during the follow-up. Compared with the SSc-ILD and other-SSc, in SSc-CPFE there was a higher prevalence of males, lower anticentromere antibodies prevalence and a more reduced pulmonary function (p<0.05). The Kaplan-Meier survival analysis demonstrates a significantly worse survival in patients with SSc-CPFE (HR vs SSc-ILD, vs SSc-emphysema and vs other-SSc, respectively 1.6 (CI 0.5 to 5.2), 1.6 (CI 0.7 to 3.8) and 2.8 (CI 1.2 to 6.6).Conclusions CPFE increases the mortality risk in SSc along with a highly impaired lung function. These findings strengthen the importance to take into account emphysema in patients with SSc with ILD.https://rmdopen.bmj.com/content/5/1/e000820.full |
| spellingShingle | Carlo Alberto Scirè Alarico Ariani Mario Silva Elena Bravi Simone Parisi Marta Saracco Fabio De Gennaro Cristian Caimmi Francesco Girelli Maria De Santis Alessandro Volpe Federica Lumetti Vanessa Hax Markus Bredemeier Veronica Alfieri Daniele Santilli Flavio Cesare Bodini Gianluca Lucchini Flavio Mozzani Valeria Seletti Emanuele Bacchini Eugenio Arrigoni Dilia Giuggioli Rafael Chakr Luca Idolazzi Giuseppina Bertorelli Davide Imberti Emanuele Michieletti Giuseppe Paolazzi Enrico Fusaro Alfredo Antonio Chetta Nicola Sverzellati Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis RMD Open |
| title | Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis |
| title_full | Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis |
| title_fullStr | Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis |
| title_full_unstemmed | Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis |
| title_short | Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis |
| title_sort | overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis |
| url | https://rmdopen.bmj.com/content/5/1/e000820.full |
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