Highlights From the Updated Clinical Practice Guideline for Progressive Pulmonary Fibrosis

Ross Summer, MD, professor of medicine, director of interstitial lung disease program, section chief of allergy, pulmonary and critical care medicine at Thomas Jefferson University, highlights the main takeaways from the 2022 updated guideline for idiopathic pulmonary fibrosis and progressive pulmonary fibrosis, including the conditional recommendation for nintedanib.

This interview is a part of the video series “Inside Interstitial Lung Disease: Overall Burden, Therapy Options, & Looking Ahead.”

Read the full transcript:

Hi everyone. I'm Ross Summer and I'm a pulmonary and critical care physician here at Thomas Jefferson University in Philadelphia, and I'm also the director of the Interstitial Lung Disease Program.

Can you please highlight the main takeaways from the latest clinical practice guidelines for idiopathic pulmonary fibrosis and progressive pulmonary fibrosis?

The 2022 guidelines were an update from earlier guidelines that were in 2011 and 2018 on idiopathic pulmonary fibrosis. These walked physicians through the management and the treatment of a specific fibrosing condition called IPF or idiopathic pulmonary fibrosis. In 2022, these guidelines were updated to include a new category of fibrosing conditions called progressive pulmonary fibrosis. And the reason for these updates was because there were several landmark studies that showed conditions that progress can also be treated by some of the medications that are used to treat idiopathic pulmonary fibrosis.

While there were various small changes to the guidelines in 2022, the major change to the guideline was the addition of the term progressive pulmonary fibrosis. And this is referring to conditions other than pulmonary fibrosis that go on and progress. Progression is defined as 2 of the following 3 things. You can have symptomatic progression in which people feel more short of breath. You can have physiologic progression in which your pulmonary function tests decline, and they gave specific criteria for that—which was a change in your forced vital capacity of 5% or more, or a change in your DLCO, which is your lung diffusion capacity for carbon monoxide of 10% or more. Or lastly, you could have changes on your imaging studies, which is represented as a worsening of your fibrotic condition.

If you met criteria for 2 of those 3, you were considered to have a progressive pulmonary fibrosing condition and you were a candidate for treatment just like you are for idiopathic pulmonary fibrosis.

The treatment landscape is as follows. There are 2 medications for the treatment of idiopathic pulmonary fibrosis. This includes nintedanib, which is a twice-a-day medication and pirfenidone, which is given 3 times a day. However, in progressive pulmonary fibrosis—so these are conditions that progress but are not idiopathic pulmonary fibrosis—there is one treatment recommendation and that is nintedanib, the twice-a-day medication. Just to reiterate, both of these medications, nintedanib and pirfenidone, are well-tolerated, and although there are side effects noted in clinical trials mostly related to GI side effects, these can be well ameliorated in clinic with simple interventions and changing dietary intake.

One important point to mention about the treatment of idiopathic pulmonary fibrosis is that in around 30% of patients, I don't care how good you are or what institution you're being managed at, there's about 30% of the time that that condition is misdiagnosed, and that's because some other conditions will present themselves later. For example, in autoimmune diseases, sometimes the first manifestation of these disease is in the lung and only later do you develop joint disease. You're given the initial diagnosis of idiopathic pulmonary fibrosis to later have rheumatoid arthritis or some other autoimmune disease. In these scenarios, when we are unsure of the diagnosis, we often start with nintedanib since that medication has been shown to be effective in various types of progressive pulmonary fibrosis conditions, IPF as well as others. Our upfront treatment is often that medication just in case we have the diagnosis wrong.

This transcript has been edited for clarity.