Format

Send to

Choose Destination
Respirology. 2020 Mar 19. doi: 10.1111/resp.13805. [Epub ahead of print]

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

Author information

1
Dipartimento Scienze Gastroenterologiche, Endocrino-Metaboliche e Nefro-Urologiche, UOC Pneumologia, Fondazione Policlinico Universitario "A.Gemelli" IRCCS, Rome, Italy.
2
Unità di Fisiopatologia Respiratoria ed Endoscopia Toracica, Policlinico Universitario Campus Bio-Medico, Rome, Italy.
3
Centre for Rare Lung Disease, University Hospital of Modena, Modena, Italy.
4
Department of Clinical and Experimental Medicine, University of Catania, Regional Referral Center for Rare Lung Diseases, A.O.U. 'Policlinico - Vittorio Emanuele', Catania, Italy.
5
University Hospital 'San Gerardo' ASST, University of Milano Bicocca, Milan, Italy.
6
Universita Cattolica del Sacro Cuore, Rome, Italy.

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.

KEYWORDS:

diagnosis; interstitial lung disease; lung fibrosis; pulmonary fibrosis

PMID:
32190952
DOI:
10.1111/resp.13805

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center