• 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, Carlo Alberto Scirè, and Nicola Sverzellati.
• Department of Medicine, Internal Medicine and Rheumatology Unit, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy.
• RMD Open. 2019 Jan 1; 5 (1): e000820.

ObjectivesThis 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).MethodsChest 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.ResultsWe 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).ConclusionsCPFE 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.

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