Idiopathic pulmonary fibrosis (IPF) is just one of many types of interstitial lung disease (ILD). There’s a lot of information out there, and it can be daunting and sometimes scary. Here are just 5 of the things that I think it’s important to know about IPF.

1. While “idiopathic” means “without a known cause”, a lot is known about risk factors for development of IPF. This includes family history, environmental pollution, certain occupational exposures, and cigarette smoking. Additionally, many of the molecular mechanisms that lead to development of IPF have been described by scientists and relate to microscopic injuries to the lung tissue followed by an inability of the lung to properly repair itself.
2. The most important information in making a diagnosis of IPF is clinical information, such as a thorough medical history, physical exam, and lab work, and a specialized x-ray called a high-resolution CT scan (HRCT). While some patients need to have a lung biopsy to confirm the diagnosis, most do not.
3. There are currently two FDA-approved drug treatments for IPF, pirfenidone and nintedanib. We refer to these medications as “antifibrotic” agents, and they have been shown in phase 3 studies to slow disease progression as measured by forced vital capacity (FVC), a pulmonary function test. Both medications are also thought to reduce risk of mortality. While both medications can cause side effects, your care team can help with ways to manage any issues if they arise.
4. Treatment for IPF goes beyond antifibrotic medications. Your pulmonologist might recommend you use oxygen or start a pulmonary rehabilitation class. Management of symptoms is also an important part of treatment for IPF.
5. There are many new therapies for IPF in development now, and volunteers are needed to participate in clinical trials that are testing these new treatments. Clinical trial participation should be considered as a care option alongside standard of care therapy.
   

Ready to learn more? Check out our PF Basics modules or tune in to a webinar to get more in depth. 

About the Pulmonary Fibrosis Foundation
At the Pulmonary Fibrosis Foundation, we are dedicated to making a difference in the lives of those affected by pulmonary fibrosis (PF), a form of interstitial lung disease (ILD). Pulmonary fibrosis is a process that causes lung scarring, in which fibrotic tissue blocks the movement of oxygen from inside the tiny air sacs in the lungs into the bloodstream. Low oxygen levels, and the stiff scar tissue itself, can cause people with pulmonary fibrosis to feel short of breath, particularly when walking and exercising. Over 250,000 Americans are living with PF today. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 Americans die from idiopathic pulmonary fibrosis (IPF) each year.
As the largest organization committed to raising awareness and providing support, our mission is to accelerate the development of new treatments and ultimately a cure for pulmonary fibrosis. Until this goal is achieved, the PFF is committed to advancing improved care of patients with PF and providing unequaled support and education resources for patients, caregivers, family members, and healthcare providers.