TY - JOUR T1 - Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis JF - RMD Open JO - RMD Open DO - 10.1136/rmdopen-2018-000820 VL - 5 IS - 1 SP - e000820 AU - Alarico Ariani AU - Mario Silva AU - Elena Bravi AU - Simone Parisi AU - Marta Saracco AU - Fabio De Gennaro AU - Cristian Caimmi AU - Francesco Girelli AU - Maria De Santis AU - Alessandro Volpe AU - Federica Lumetti AU - Vanessa Hax AU - Markus Bredemeier AU - Veronica Alfieri AU - Daniele Santilli AU - Flavio Cesare Bodini AU - Gianluca Lucchini AU - Flavio Mozzani AU - Valeria Seletti AU - Emanuele Bacchini AU - Eugenio Arrigoni AU - Dilia Giuggioli AU - Rafael Chakr AU - Luca Idolazzi AU - Giuseppina Bertorelli AU - Davide Imberti AU - Emanuele Michieletti AU - Giuseppe Paolazzi AU - Enrico Fusaro AU - Alfredo Antonio Chetta AU - Carlo Alberto Scirè AU - Nicola Sverzellati Y1 - 2019/02/01 UR - http://rmdopen.bmj.com/content/5/1/e000820.abstract N2 - 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. ER -