RT Journal Article
SR Electronic
T1 Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis
JF RMD Open
JO RMD Open
FD EULAR
SP e000820
DO 10.1136/rmdopen-2018-000820
VO 5
IS 1
A1 Alarico Ariani
A1 Mario Silva
A1 Elena Bravi
A1 Simone Parisi
A1 Marta Saracco
A1 Fabio De Gennaro
A1 Cristian Caimmi
A1 Francesco Girelli
A1 Maria De Santis
A1 Alessandro Volpe
A1 Federica Lumetti
A1 Vanessa Hax
A1 Markus Bredemeier
A1 Veronica Alfieri
A1 Daniele Santilli
A1 Flavio Cesare Bodini
A1 Gianluca Lucchini
A1 Flavio Mozzani
A1 Valeria Seletti
A1 Emanuele Bacchini
A1 Eugenio Arrigoni
A1 Dilia Giuggioli
A1 Rafael Chakr
A1 Luca Idolazzi
A1 Giuseppina Bertorelli
A1 Davide Imberti
A1 Emanuele Michieletti
A1 Giuseppe Paolazzi
A1 Enrico Fusaro
A1 Alfredo Antonio Chetta
A1 Carlo Alberto Scirè
A1 Nicola Sverzellati
YR 2019
UL http://rmdopen.bmj.com/content/5/1/e000820.abstract
AB 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.