Idiopathic pulmonary fibrosis (IPF) is a rare and serious irreversible lung disease. ‘Idiopathic’ means that the cause of the disease is unknown. ‘Pulmonary’ means it’s a disease that impacts the lungs. ‘Fibrosis’ means scarring. In all, ‘IPF’ means an unknown factor is causing scarring of the lungs.
Idiopathic pulmonary fibrosis (IPF) [ID-ee-oh-PATH-ik PUHL-mon-air-ee fi-BRO-sis]
Over time, this scarring makes it difficult for the lungs to expand as you breathe. This is what’s known as a restrictive lung disease. However, the non-specific symptoms of IPF may lead to misdiagnoses of obstructive lung diseases like chronic obstructive pulmonary disease (COPD) and asthma. Unlike IPF and other restrictive lung diseases where the lungs can’t fully expand as you inhale, obstructive lung diseases occur when there is lung impairment due to a blockage or narrowing of the airways.
If you are wondering if you may have IPF, you may want to bring this information with you when you see your family doctor.
IPF by the Numbers
- There are 30,000 to 40,000 new cases each year
- Usually affects people at least 50 years old
- Over 50% of cases are misdiagnosed at first
Diagnosing and Managing IPF
Diagnosing IPF can be a long and difficult process. In fact, over 50 percent of IPF cases are misdiagnosed at first. That’s because IPF is such a rare disease and its symptoms are easily confused for other conditions like COPD, cardiac disease, or simply aging.
However, early diagnosis of IPF is important22 because it is a progressive disease. That means the condition will worsen over time — sometimes very quickly. While there is no cure for IPF, there are treatments available that may help slow the progression of the disease. The sooner you receive a diagnosis, the sooner you can work with your family doctor to learn about your treatment options.
Your family doctor will likely use several tests and tools to make a diagnosis including special chest x-rays known as high-resolution computed tomography (HRCT) and lung function tests. A lung biopsy (obtaining lung tissue) is sometimes necessary. Your family doctor may refer you to a pulmonologist. A pulmonologist is a doctor that specializes in treating lung diseases, and likely has the expertise required to diagnose and treat IPF.
IPF can be an overwhelming and isolating disease. But it’s important to remember that you’re not alone. It may be helpful for you to connect with other people who are living with IPF. Plus, your family doctor can offer community information and continued support as you manage life with IPF.
Learn more about possible IPF risk factors
Copyright © American Academy of Family Physicians
This information provides a general overview and may not apply to everyone. Talk to your family doctor to find out if this information applies to you and to get more information on this subject.