Disease progression across the spectrum of idiopathic pulmonary fibrosis: A multicentre study

Abstract

Background and objective: In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB. In this study, we compared the disease course across IPF diagnostic categories in a real-life clinical setting to clarify the appropriateness of a working diagnosis of IPF and treatment initiation in these patients. Methods: Longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) were retrospectively collected at three tertiary centres in Italy. Univariate and multivariate analyses were performed to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups, that is, patients with UIP on HRCT and/or SLB, and patients with possible UIP and no histological confirmation. Results: A total of 249 IPF patients were included in the analysis. Among patients with a possible UIP pattern on HRCT, 41 (55%) were prescribed antifibrotic treatment (either nintedanib or pirfenidone) despite absence of histological confirmation. This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR: 1.19, 95% CI: 0.49-2.89, P = 0.7 for death; HR: 1.42, 95% CI: 0.83-2.44, P = 0.201 for disease progression). Conclusion: In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of IPF according to consensus guidelines, supporting the rationale for treatment initiation in these patients by expert multidisciplinary teams.

Contemporary Concise Review 2020: Interstitial lung disease

“…While these studies provide compelling data to support the utility of TBLC integration into ILD diagnostic algorithms, the utility of lung biopsies in the diagnosis of IPF is less clear. A retrospective multicentre analysis between 2011 and 2017 showed that more than half of the patients with a possible UIP pattern on HRCT were labelled and treated as IPF with antifibrotic agents, suggesting a general tendency of clinicians avoiding invasive procedures 18 . More importantly, the study showed that patients with a possible UIP pattern on HRCT without histopathological confirmation had similar mortality and disease progression compared to those with a definite diagnosis of IPF.…”

The Effectiveness of Nintedanib in Patients with Idiopathic Pulmonary Fibrosis, Familial Pulmonary Fibrosis and Progressive Fibrosing Interstitial Lung Diseases: A Real-World Study

“…Notably, the improvement of survival rate and progression-free survival was observed across different clinical (age, sex, respiratory functional parameters) and/or diagnostic subgroups (confident or working diagnosis) of patients affected with IPF [ 12 , 13 ]. However, no specific trials have been conducted for the evaluation of nintedanib efficacy in patients with familial pulmonary fibrosis (FPF), even though FPF is estimated to represent at least 10% of IPF [ 14 ].…”

Diagnosing idiopathic pulmonary fibrosis: Has the time for surgical lung biopsy passed?

“…Laura M. Glenn, BMed, MD1,2,3 and Tamera J. Corte, BSc (Med), MBBS (Hons), FRACP, PhD1,2,3 1 Interstitial Lung Diseases Unit, Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia; 2 The University of Sydney School of Medicine (Central Clinical School), Sydney, NSW, Australia; 3 NHMRC Centre of Research Excellence in Pulmonary Fibrosis, Sydney, NSW, Australia…”

“…In a recent publication in Respirology , Sgalla et al . retrospectively compared the rates of mortality and disease progression in 249 IPF patients receiving anti‐fibrotic treatment across three centres in Italy between two diagnostic subgroups: patients with definite UIP on HRCT or SLB and patients with possible UIP on HRCT and no SLB 3 . The major finding of this study was that patients with possible UIP demonstrated similar mortality (log‐rank test P = 0.771) and disease progression (log‐rank test P = 0.139) as compared to patients with UIP on HRCT or SLB.…”