Patients with idiopathic pulmonary fibrosis (IPF) may benefit from Esbriet (pirfenidone) treatment regardless of their disease stage and baseline lung function, according to a study by researchers at the University of Turin, in Italy, and colleagues.
The study, “Efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis with more preserved lung function,” was published in the European Respiratory Journal.
Current treatment options for IPF, such as Esbriet and Ofev/Vargatef (nintedanib), can delay disease progression but are not curative. It is speculated that treating patients early in the disease course may be the best strategy to preserve functional status and extend a patient’s life, but no studies to support the hypothesis had been published.
The researchers aimed to examine the effectiveness of Esbriet, an oral antifibrotic agent with anti-inflammatory properties, in distinct stages of the disease. Esbriet had already been shown to decrease disease progression in IPF, as assessed by progression-free survival, lung function, and tolerance to exercise, but whether it was more efficient when administered to patients in early IPF stages, was to be determined.
In data pooled from 1,247 patients included in three Phase 3 studies: the CAPACITY (NCT00287716 and NCT00287729), and ASCEND (NCT01366209) trials, Esbriet (2,403 mg per day) was shown to be well-tolerated and to significantly decrease all-cause mortality and IPF-related mortality at one year compared to placebo.
In the recent review, researchers stratified the patients by baseline forced vital capacity (FVC) or GAP (gender, age, physiology) index, two distinct measures of disease progression in patients with IPF. Among the patients, 316 had FVC of 80 or higher, and 931 had a FVC lower than 80. On the other hand, when patients were stratified using the GAP index stage, which also takes into account a patient’s age and gender, 482 were included in the GAP stage I, and 736 in the GAP stage II-III.
Overall, patients were found to have clinically significant disease progression over 12 months, independent of having a more preserved or less preserved lung function at baseline, as assessed by declines in FVC, six-minute walking distance (6MWD), and shortness of breath scores. But patients with FVC lower than 80 were found to be 67 percent more likely of decline in the 6MWD and nearly three times more likely to experience a decline in shortness of breath scores than those with an FVC of 80 or higher. Similar observations were found when the patients were stratified with the GAP index score.
Of significance, Esbriet-treated patients were found to be less likely to experience decline in any of the tests — true for patients included in all GAP index scores and for those with FVC lower than 80 — but not for patients whose FVC was 80 or higher.
Researchers concluded: “These findings support the initiation of treatment with pirfenidone, irrespective of stage of baseline lung function in this patient population.”