Nintedanib and pirfenidone in patients with FVC <50%

What is your opinion on using nintedanib and pirfenidone in patients with forced vital capacity (FVC) <50%? Are there any data available to support such treatment?

Luca Richeldi: Patients with an FVC below 50% have not been enrolled in clinical trials, so this population has not been tested for the safety and efficacy of these drugs. That is the first message. As far as we know, we do not have a reassuring answer for these patients.

However, we have a reassuring answer for patients with an FVC above 50%. Idiopathic pulmonary fibrosis (IPF) is a progressive disease, so every patient that achieved an FVC below 50% before had been on an FVC above 50%. There are no reasons to think that a patient with an FVC below 50% will be different in terms of efficacy, but we are not sure about the safety. That is why most guidelines do not recommend treatment for patients with an FVC below 50%. It is also true that in countries like the US, the Food and Drug Administration (FDA) approved a drug for patients with any FVC value.

It is still an area in which there is debate. It is an evolving concept. We hope to have more placebo-controlled [trials] in the severe population, but for the time being, we cannot give a definitive answer to that question in the case of patients with severe disease.