Interstitial lung disease

Some blood tests can help identify other conditions or causes of inflammation (such as autoimmune disease) that are causing symptoms or scarring the lungs.

A computed tomography (CT) scan is a series of X-rays taken at different angles and combined on a computer. At Penn, we use high-resolution CT technology to take very detailed pictures of your lungs. These clear images can show the location and severity of any abnormalities, which helps us make the most accurate diagnosis.

Our cardiothoracic imaging specialists have extensive expertise interpreting lung CT scans. Based on your symptoms, exam findings, bloodwork and CT images, we can often make an accurate ILD diagnosis without surgery to test the lung tissue.

To measure how well your lungs are working, your team may perform pulmonary function tests, such as:

• Exercise test: During this pulmonary function test, you walk at your normal pace for six minutes. Your heart rate, oxygen saturation, and blood pressure, and the distance you walked are all recorded.
• Spirometry, lung volumes and diffusion capacity: This test measures how much air you can breathe and how easily and quickly you can blow the air out. Your team will ask you to exhale into a tube connected to a machine.

There are sometimes different options to obtain a diagnosis, so we will seek your input to determine the right path for you. And our specialists often present cases at an ILD multidisciplinary conference to discuss the best approach. The team includes pulmonology physicians, thoracic radiologists and lung pathologists. This helps ensure that an invasive procedure is performed only when necessary.

If we can't diagnose ILD from imaging alone, you may need a biopsy. In this procedure, your doctor takes a small sample of tissue from your lung and sends it to a lab.

There are a few ways the team can take a bit of tissue for testing. We use the least invasive method possible to avoid complications and keep you more comfortable.

• Bronchoscopy: For this procedure, an expert interventional pulmonologist inserts a thin, flexible tube through your mouth or your nose. They advance the tube into your lung and collect a tiny sample of tissue. We also may use advanced techniques such as cryobiopsy and genomic testing to make a more accurate diagnosis.
• Surgical biopsy: To get a larger piece of tissue, your team may recommend surgery. You will be under general anesthesia while a Penn thoracic surgeon makes several small incisions in your chest near your lungs. The surgeon uses a camera to guide a surgical instrument into your lung and remove a tissue sample.

After a biopsy, a pathologist specialized in ILD looks at the sample for signs of disease and the multidisciplinary team reviews the report to make the most accurate diagnosis.

ILD with no known cause is called idiopathic pulmonary fibrosis (IPF). If you have IPF, your doctor may recommend one of two drugs that slow down certain types of scarring: pirfenidone (Esbriet®) and nintedanib (Ofev®).

If your doctor prescribes pirfenidone or nintedanib, you will have regular bloodwork to monitor your liver function.

Pulmonary rehabilitation is exercise in a safe environment designed to reduce ILD symptoms, increase lung function and improve quality of life.

Our on-site pulmonary rehabilitation centers are staffed by respiratory therapists, physical therapists, occupational therapists and dieticians with in-depth experience helping patients with ILD. We can also help connect you to pulmonary rehabilitation facilities closer to you.

This specialized rehabilitation can show you:

• How to use oxygen supplementation
• Methods to conserve energy doing everyday activities, including your setup at home
• Safe ways to exercise
• Techniques to relax when you have trouble breathing
• Tips to improve your nutrition, which will boost health and energy levels
• Ways to breathe to improve lung efficiency

Oxygen therapy gives your body extra oxygen to make breathing easier. It also protects the rest of your body from complications of low oxygen. It can improve your overall health and give you more energy.

Your team will evaluate your symptoms and lifestyle to help you decide whether you need supplemental oxygen. They'll also educate you about how to safely and effectively use and store oxygen.

Steroids are medications that can lessen inflammation in the lungs. Corticosteroid therapy is useful for some patients with ILD. However, long-term steroid use can cause other problems.

Our expert pulmonologists work with you to decide whether steroids are the right choice for you. We have extensive experience using "steroid-sparing" medications that can control inflammation without the use of steroids.

Some people with advanced ILD may need a lung transplant. The Penn Lung Transplant program is among the best in the world.

We introduce patients to the transplant team imminently, long before a transplant is needed. This helps teach you about the process slowly so that you know what to expect. It also helps the transplant team identify any issues that need to be addressed before a lung transplant procedure, such as losing weight or establishing a support system.