Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a disease that leads to the accumulation of scar tissue in the lungs. When the scar tissue develops between the air spaces and blood stream, it makes it very hard for the body to absorb oxygen and create energy. Of all the interstitial lung diseases, IPF is the most common.

Symptoms of IPF include:

• Shortness of breath during exertion
• Chronic cough

IPF is not the only disease that can cause scarring of the lung. Before you can be diagnosed with IPF, your physician must complete a thorough search for other causes of scarring. This typically includes a careful medical history, occupational history, physical exam and blood work. When no other reason for lung scarring can be found, your physician will study the pattern of scarring through the results of a CAT scan. If the pattern is typical for IPF, we can often be confident of the diagnosis. If the CAT scan pattern isn’t typical we may need additional testing such as a lung biopsy to be certain.

Once a detailed diagnosis is reached, your physician will determine the best treatment options for you.

IPF is a progressive condition, meaning that it gets worse over time. However, the progress of your disease can vary a great deal. Some patients worsen quickly, but that isn’t typical. Many patients will have a “stair step” progression – meaning there are periods of stability followed by a drop in function followed by another period of stability. At Penn Medicine, we work with each patient individually, tailoring the care on a case-by-case basis. Our specialists are experts in the most innovative treatments available for IPF, including the following:

• IPF specific therapy: In 2014, two drugs were approved for use in the treatment of IPF, pirfenidone and nintedanib. Both drugs slow disease progression but don’t improve function or even stop decline. Both drugs have potential side effects and you will require ongoing monitoring by your physician.
• Oxygen: The “roadblock” that scar tissues form between air spaces and the bloodstream, many patients with IPF need oxygen at least with exercise and sleep. Oxygen therapy allows more oxygen into the bloodstream, allowing you to do more of the things you enjoy while protecting your heart from damage.
• Pulmonary rehabilitation: This is a supervised exercise program for patients with lung disease. Rehabilitation programs allow patients to remain more active and feel better with the same level of lung disease.
• Symptom therapy: Our physicians can create detailed, individual treatment plans to help alleviate the symptoms of IPF such as cough, shortness of breath, heartburn, anxiety, and depression.
• Lung transplantation: For eligible patients with significant progression of their disease, a lung transplant may be an appropriate option.

If your condition requires more intervention, such as lung surgery, know that Penn is a national leader in lung transplantation. Penn Medicine’s specialized surgeons offer medical treatments not available at other centers and perform four times as many lung transplants as any other regional hospital. Penn specialists excel in procedures in chest, lung, and artificial breathing operations, outperforming any other area hospital.

When you choose Penn, you choose a dedicated team offering the most innovative treatments and surgeries. Penn physicians continue to lead the field, advancing the science of lung care.