Survival and mortality associated factors in progressive fibrosing interstitial disease

Abstract

<b>Background:</b> Unlike other ILDs, progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) seem to share similar progression phenotype. We aimed to assess Survival and mortality associated factors in PF-ILD. <b>Methods:</b> Data of patients with PF-ILD, treated in Charles Nicolle Hospital of Tunis, from 2001 to 2019, whose last visit was in December 2021, were retrospectively analysed. Baseline characteristics and clinical outcomes were collected. <b>Results:</b> Among 121 patients with ILD, 34 patients (28%) met the diagnostic criteria for FP-ILD (excluding IPF). There were 11 (32.4%) cases of connective (CTD-ILD) and 11 (32.4%) of sarcoidosis. Kaplan-Meier survival curves revealed that the 2 and 5-years survival of PF-ILD were 82.4 and 41.2%, respectively. The PF-ILD group also showed poorer survival (p&lt;0.001) than the non-PF-ILD group, but a similar survival with IPF (p=0.49). According to subgroups of PF-ILD, Kaplan-Meier survival curves, didn’t showed a significant difference between groups (p=0.73), although CTD-ILD seems to have a lower mortality 2-years survival (18% vs 33%). Survival analysis revealed that the dyspnoea mMRC4 (p=0.05), time from the diagnosis of ILD to PF-ILD 12 months (p=0.05), FVC% predicted &lt;50% (p=0.04), polynuclear neutrophilic alveolitis (p=0.001), eosinophilic alveolitis (p=0.007) and high CD4/CD8 ratio (p=0.009), were all associated factors with the mortality of PF-ILD. However, Extent of fibrotic changes on HRCT and pulmonary hypertension weren’t predictors of mortality in patients with PF-ILD. <b>Conclusion:</b> Through our study, FP-ILD and IPF share the same survival profile and have worse prognosis than in non FP-ILD patients.