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Maria Grazia DE SANTIS
Personale tecnico amministrativo
Scuola di Ateneo Facoltà di Medicina e Chirurgia
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Pubblicazioni
2019
- Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis
[Articolo su rivista]
Ariani, A.; Silva, M.; Bravi, E.; Parisi, S.; Saracco, M.; De Gennaro, F.; Caimmi, C.; Girelli, F.; De Santis, M.; Volpe, A.; Lumetti, F.; Hax, V.; Bredemeier, M.; Alfieri, V.; Santilli, D.; Bodini, F. C.; Lucchini, G.; Mozzani, F.; Seletti, V.; Bacchini, E.; Arrigoni, E.; Giuggioli, D.; Chakr, R.; Idolazzi, L.; Bertorelli, G.; Imberti, D.; Michieletti, E.; Paolazzi, G.; Fusaro, E.; Chetta, A. A.; Scire, C. A.; Sverzellati, N.
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 χ 2 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.