Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis. Issue 1 (14th February 2019)

• Record Type: Journal Article
• Authors: Ariani, Alarico; Silva, Mario; Bravi, Elena; Parisi, Simone; Saracco, Marta; De Gennaro, Fabio; Caimmi, Cristian; Girelli, Francesco; De Santis, Maria; Volpe, Alessandro; Lumetti, Federica; Hax, Vanessa; Bredemeier, Markus; Alfieri, Veronica; Santilli, Daniele; Bodini, Flavio Cesare; Lucchini, Gianluca; Mozzani, Flavio; Seletti, Valeria; Bacchini, Emanuele; Arrigoni, Eugenio; Giuggioli, Dilia; Chakr, Rafael; Idolazzi, Luca; Bertorelli, Giuseppina; Imberti, Davide; Michieletti, Emanuele; Paolazzi, Giuseppe; Fusaro, Enrico; Chetta, Alfredo Antonio; Scirè, Carlo Alberto; Sverzellati, Nicola;
• Is Part Of: RMD open. Volume 5:Issue 1(2019)
• Publication Date: 2019-02-14
• Subjects: pulmonary fibrosis -- combined pulmonary fibrosis and emphysema -- systemic sclerosis -- semiquantitative chest CT; Musculoskeletal system -- Diseases -- Periodicals; Rheumatism -- Periodicals; 616.7005
• DOI: 10.1136/rmdopen-2018-000820
• Languages: English
• ISSNs: 2056-5933

Abstract

Abstract : 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.