Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis

Abstract

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.

Combined Pulmonary Fibrosis and Emphysema

“…However, Ariani et al recently demonstrated that CPFE increases the mortality risk in SSc along with a highly impaired lung function. 52 The main determinant of prognosis in CPFE is represented by pulmonary hypertension which develops in 30 to 50% of patients with CPFE. 52…”

“…52 The main determinant of prognosis in CPFE is represented by pulmonary hypertension which develops in 30 to 50% of patients with CPFE. 52…”

Computed Tomography Predictors of Mortality or Disease Progression in Systemic Sclerosis–Interstitial Lung Disease: A Systematic Review

“…Out of 3,513 papers identified, 15 fulfilled inclusion criteria, for a total amount of 2,332 patients with SSc (range 21-595 patients per paper) (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22). The detailed flow diagram of studies' selection is available in Figure 1.…”

“…Papers that were not included because only partially meeting inclusion criteria, but relevant for the discussion, are listed in Supplementary Data 1, with the reason of rejection. All included studies were retrospective (8)(9)(10)(11)(12)(13)(14)(15)(16)(18)(19)(20)(21)(22) or retrospective analyses of prospectively collected data (17) and adopted CT for ILD diagnosis (8-19, 21, 22), except for one that utilized CT or chest X-ray (20). Prone acquisitions were additionally performed to confirm ILD presence in one study (12), while in one work prone scans could be part of the protocol, but only supine CT was analyzed (19).…”

Quantitative Computed Tomography in Systemic Sclerosis–Interstitial Lung Disease: Are We Ready to Go beyond Standard Assessment?

“…However observational data suggest that in the SSc, emphysema is associated with ILD, appearing as a combined pulmonary fibrosis and emphysema syndrome ( 3 ). Having excluded these patients who have a poor prognosis could have depleted the cohort of patients at a high risk of mortality ( 4 ). A second limitation that Saldana and colleagues report is the scarce possibility of using QCT in clinical routine for the following two reasons: inability to process computed tomographic (CT) scans of all patients (38% of all CT scans performed) and the significant time required to evaluate each CT scan.…”