Idiopathic pulmonary fibrosis
What is idiopathic pulmonary fibrosis (IPF)?
Idiopathic pulmonary fibrosis, or IPF, causes scar tissue to build up in the lungs. As scar tissue accumulates, the lungs stiffen, making it difficult to breathe. This results in inadequate oxygen being delivered to the rest of the body.
While IPF is a progressive lung disease that can get worse over time, the rate of progression can vary a great deal in different people. Some people may have a rapid progression, while others will have a “stair-step” progression where there are periods of stability followed by a drop in function, followed by another period of stability.
At Penn Medicine, we work with each person individually, tailoring chronic lung disease care and treatment plans on a case-by-case basis. You’ll receive care from our expert pulmonologists who can help slow the progression of IPF and manage any symptoms.
Symptoms of idiopathic pulmonary fibrosis
If you have IPF, your symptoms can include:
- Shortness of breath during exertion
- A chronic dry or hacking cough
- Breathing that is faster or more shallow than usual
- Unexpected weight loss
- Unusual fatigue
- Joint and muscle aches
- Changes in the shape of nails or in the tips of toes or fingers
Known causes of IPF
IPF is the most common type of pulmonary fibrosis, a disease that causes thickening or scarring of the lungs and leads to stiff lungs. Unlike other types of pulmonary fibrosis, IPF has no known cause. This fibrosis of the lungs is generally more common in people assigned male at birth though can occur in anyone. Symptoms often appear between the ages of 50 and 70.
How is idiopathic pulmonary fibrosis diagnosed?
To diagnose IPF, your doctor will ask about your medical and occupational history, perform a physical exam, and analyze blood work to evaluate you for signs of the disease. They’ll also use imaging tests to assess the visual pattern of lung scarring that is typical for IPF. Additional testing may be required to confirm the diagnosis.
IPF treatment
Our specialists are experts in innovative treatments for IPF. This includes medications that slow the progression of the disease or treat symptoms. Different types of outpatient therapy, including pulmonary rehabilitation, are often useful. Physical therapy can help you stay active, while cognitive behavioral therapy provides support to those with anxiety and depression. Other treatments are available depending on the extent of your IPF progression and overall health.
Experts in chronic lung disease care
Penn Medicine specialists offer extensive experience in chest, lung, and artificial breathing procedures, including treatment for IPF lung disease. Here, you’ll benefit from coordinated care across disciplines. Our pulmonologists partner with radiologists, pathologists, and transplant specialists to ensure that you receive the right care from the right experts. This is why more people seek our pulmonary care than any other hospital in the greater Philadelphia, Lancaster, and Princeton regions.
For those who are candidates for organ transplantation, our Transplant Institute is a national leader in lung transplant care. Our surgeons perform four times as many lung transplants as any other hospital in the southeastern Pennsylvania and New Jersey regions and offer medical treatments not available at other centers.
When you choose Penn Medicine for lung care, you choose to work with a dedicated team offering the most innovative treatments and surgeries. Our doctors are leaders in chronic lung disease care and work side-by-side with researchers to advance the science of lung care while providing you with individualized care.