Disease progression rates similar between patients with working, definite IPF diagnosis

Patients receiving antifibrotic medication following a working diagnosis of idiopathic pulmonary fibrosis (IPF) show similar disease progression rates to those with a confident IPF diagnosis according to consensus guidelines, a study has shown.

This finding supports the rationale for treatment initiation in patients with a working diagnosis of IPF by expert multidisciplinary teams.

The authors retrospectively collected longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) at three tertiary centres in Italy. They performed univariate and multivariate analyses to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups (patients with usual interstitial pneumonia [UIP] on high-resolution computed tomography (HRCT) and/or surgical lung biopsy [SLB] and those with possible UIP and no histological confirmation).

In total, 249 IPF patients were included in the analysis. Forty-one patients (55 percent) among those with a possible UIP pattern in HRCT were administered antifibrotic treatment despite the absence of any histological confirmation. As a result, these patients showed similar mortality and disease progression to those with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test: p=0.771 and p=0.139, respectively).

Multivariate analysis confirmed these findings (hazard ratio [HR] for death, 1.19, 95 percent confidence interval [CI], 0.49–2.89; p=0.7; HR for disease progression, 1.42, 95 percent CI, 0.83–2.44).

“In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB,” the authors said.