Patients with idiopathic pulmonary fibrosis (IPF) who demonstrate adequate lung volume still have the same rate of lung volume decline as patients with initially lower volumes, and benefit equally from treatment with Ofev (nintedanib), researchers reported.
The study, “Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume,” published in the journal Thorax, is likely to disperse prevailing doubts about treating pulmonary fibrosis patients with preserved lung volume.
Although much research has focused on improving treatment in lung fibrosis, there is no consensus among researchers and clinicians about how to describe the severity of the disease, and when to start treatment. Fibrosis is commonly described as mild, moderate, and severe, or early and advanced, but there are no standardized definitions of what a mild disease might mean. Some national guidelines state that treatment should be started at a particular cut-off value of predicted forced vital capacity (FVC), but researchers at McMaster University in Canada noted that such guidelines are not based on scientific evidence.
In collaboration with Boehringer Ingelheim, the researchers set out to analyze data from the two Phase 3 INPULSIS trials (NCT01335464 and NCT01335477) evaluating Ofev treatment in IPF. The team compared patients in the trials who had a lung volume of either more or less than 90 percent predicted FVC.
Analysis showed that patients with a lung volume of more than 90 percent predicted FVC had been sick for a shorter time, had a better lung function, and scored better on a questionnaire measuring disease impact on overall health, daily life, and perceived well-being.
At study start, 274 patients had an FVC of more than 90 percent, and 787 patients an FVC of 90 percent or less. Among patients receiving placebo, the decline in FVC was nearly identical in patients with high and low lung volume: −224.6 mL/year and −223.6 mL/year, respectively.
The two groups also did not differ in how many patients discontinued treatment before the study finished.
When looking only at patients treated with Ofev, there was no difference in the rate of lung volume decline, which was −91.5 mL/year in those with better volume at study start, and −121.5 mL/year in patients with poorer initial volume. The difference from placebo was 133.1 mL/ year and 102.1 mL/year, respectively.
Results showed that Ofev prolonged the time to first disease exacerbation in both groups. More patients in the group with poorer lung volume (less than 90 percent predicted) had more than one acute exacerbation — 5.9% compared to 1.8 percent in the group with better lung volume. This difference was not statistically significant.
“Patients with IPF and preserved lung volume (FVC >90% predicted) have the same rate of FVC decline and receive the same benefit from nintedanib as patients with more impaired lung volume,” the team concluded, adding, “These data provide, for the first time, evidence in a significant subgroup of patients with preserved lung volume to support the concept of offering early treatment to patients with IPF.”