Pulmonary fibrosis is scarring (called fibrosis) in the tissue inside the lungs. It’s just one of about 200 interstitial pulmonary diseases that affect the tissue around the balloon-like structures (called air sacs) attached to the lung airways.

Scarring in pulmonary fibrosis results from damage caused by inhaled environmental pollutants, radiation treatment, certain prescription medicines, autoimmune diseases, or genetic disorders. In many cases, there is no known cause (called idiopathic pulmonary fibrosis).

Symptoms of pulmonary fibrosis include a chronic (ongoing) dry cough, shortness of breath, chest discomfort, and weight loss. Progressive (worsening) tissue stiffness makes it hard to breathe, and lack of oxygen in the blood can lead to life-threatening conditions. Complications of pulmonary fibrosis include:

• Heart failure: caused by the right ventricle pumping blood harder than usual to move blood to the lungs
• Lung cancer: abnormal cell production in the lung
• Other lung problems: infections, blood clots, or collapsed lung
• Pulmonary hypertension: high blood pressure in the pulmonary artery
• Respiratory failure: when not enough oxygen passes from the lungs into the blood

If you have symptoms of pulmonary fibrosis, visit a pulmonologist (lung specialist) at the University of Miami Health System. We’ll manage your symptoms, so that you can live a healthier life.

Tests

Arterial blood gas (ABG)
This test measures the amount of oxygen and carbon dioxide in the blood, using a sample taken from an artery. Results show how well your lungs work, and whether you need oxygen therapy for pulmonary fibrosis.

Biopsy
A lung biopsy is a tissue sample taken from the lung and analyzed at a lab. The type of biopsy depends on your condition and may include:

• Needle lung biopsy: performed using CT (computed tomography) imaging to guide the needle to the right place
• Bronchoscopy biopsy: a thin, flexible tube is inserted in the mouth or nose to access the lung
• Surgical lung biopsies: includes a minimally invasive technique called video-assisted thoracoscopy surgery (VATS) or an open thoracic (chest) procedure to remove a wedge, a lobe, or a whole lung

Exercise stress test
Your doctor may order an exercise test to measure lung function before, during, and after physical activity, using a treadmill or stationary bike.

High-resolution computed tomography scan (HRCT)
HRCT is a special CT (computed tomography) scan that highlights thin sections of the lungs to detect pulmonary fibrosis or other forms of in interstitial pulmonary disease.

Pulmonary function tests
Pulmonary function tests measure lung functioning. They show how much air your lungs can hold, how quickly air moves in and out, and how well the carbon dioxide/oxygen gas exchange works. Methods include spirometry, or a mouthpiece attached to a machine, and plethysmography, performed in a box that looks like a phone booth.

Treatments

Medicine therapy
Oral and injected medications can decrease inflammation, suppress an overactive immune system, and slow scarring progression. These include corticosteroids, chemotherapy, and immunotherapy drugs.

Oxygen therapy
If blood oxygen levels are too low, your doctor may prescribe inhaled oxygen as needed, such as before exercise or long-term for most of the day. There are different devices available. Many are portable, meaning they’re easy to move or take with you on the go.

Pulmonary rehabilitation
Pulmonary rehabilitation programs involve support, education, exercise, and nutrition counseling and training to help you live better with COPD. Our programs are personalized to your needs, and focus on keeping you active and enjoying life.

Transplant
If your condition is severe and doesn’t respond well to other treatments, a lung transplant may be an option.

Why Choose UHealth?

Researching new and better treatments and care for pulmonary fibrosis. We’re studying medicine safety and effectiveness for idiopathic pulmonary fibrosis and other interstitial lung diseases. You can trust you’re getting the most innovative treatments, sooner. Learn more about our clinical trials.

Advanced diagnostics and nuclear imaging. With advanced diagnostic tests and imaging and interventional radiology techniques (called bronchoscopy), we can get better results, for more accurate diagnosis. This means we can start treatment early, and even slow disease progression.

Critical care experts here for you from hospital to home. Our pulmonologists are experts in pulmonary fibrosis and offer services in the hospital and intensive care unit. We also provide follow-up outpatient (clinic) appointments in convenient South Florida locations. You get seamless care from some of the same providers who know you and understand your medical history.