Pulmonary Fibrosis

Diagnosis

To diagnose pulmonary fibrosis, a healthcare professional reviews your medical and family history, conducts a physical exam, and discusses your symptoms. You may also be asked about long-term or frequent exposure to dust, chemicals, gases or other substances, especially in a work setting.

During the physical exam, the healthcare professional listens to your lungs while you breathe. Pulmonary fibrosis may cause a crackling sound at the base of the lungs.

You may undergo one or more of the following tests.

Imaging tests
Chest X-ray. A chest X-ray may show scar tissue linked to pulmonary fibrosis. Sometimes, no changes are visible, and further testing is required to determine the cause of shortness of breath.
Computerized tomography scan. A CT scan uses X-ray images from different angles to form detailed internal images. A high-resolution CT scan can help diagnose pulmonary fibrosis and show the extent of lung damage. Certain patterns may indicate specific types of fibrosis.
Echocardiogram. This test uses sound waves to create images and videos of the heart. It can show how well the heart is functioning and estimate pressure in the lung arteries and the right side of the heart.

Lung function tests
Also known as pulmonary function tests, these measure how well your lungs are working:
• Spirometry measures how much air the lungs can hold and how quickly air moves in and out.
• Lung volume tests show how much air the lungs hold at different stages of breathing.
• Lung diffusion tests measure how effectively oxygen and carbon dioxide move between the lungs and the blood.
• Pulse oximetry uses a small device on the finger to measure oxygen saturation. A six-minute walking test may be recommended to check oxygen levels during activity.
• Exercise stress tests, performed on a treadmill or stationary bike, show how the heart and lungs respond during exertion.
• Arterial blood gas tests measure oxygen and carbon dioxide levels in a blood sample taken from an artery, often in the wrist.

Imaging and lung function tests also help monitor the condition over time and track how well treatments are working.

Tissue sample
If other tests cannot determine the cause, a small lung tissue sample may be needed. This biopsy is examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. Methods include:
• Surgical biopsy, either through minimally invasive video-assisted thoracoscopic surgery or open thoracotomy. Both require general anesthesia.
• Bronchoscopy, which uses a flexible tube to collect very small tissue samples. These samples are sometimes too small for a definite diagnosis but can help rule out other conditions.

Blood tests
Blood samples may be used to check liver and kidney function and to rule out other health conditions.

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Treatment

The lung scarring and thickening caused by pulmonary fibrosis cannot be reversed. No current treatment can stop the disease from progressing, but some treatments may improve symptoms temporarily, slow disease worsening, or enhance quality of life.

Treatment plans depend on the cause of pulmonary fibrosis and the severity of the condition. Together with your healthcare professional, you can decide on the most appropriate approach.

Medicines
For idiopathic pulmonary fibrosis, medicines such as pirfenidone or nintedanib may be recommended. Both are approved by the U.S. Food and Drug Administration. Nintedanib is also approved for other rapidly worsening forms of pulmonary fibrosis. These medicines may help slow disease progression and reduce sudden symptom flare-ups.

Nintedanib may cause diarrhea and nausea. Pirfenidone may cause nausea, appetite loss, and sun-related skin rash. Regular blood tests help monitor liver function during treatment.

New therapies are being studied in clinical trials but are not yet FDA-approved. Anti-acid medicines may be recommended if you have gastroesophageal reflux disease, which is common in idiopathic pulmonary fibrosis.

Oxygen therapy
Supplemental oxygen cannot prevent lung damage, but it can:
• Make breathing and exercise easier
• Reduce complications from low oxygen levels
• Lower strain on the right side of the heart
• Improve sleep and overall well-being

You may use oxygen only during sleep or exercise, or you may need it continuously. Portable tanks and concentrators can improve mobility.

Pulmonary rehabilitation
Pulmonary rehabilitation programs help manage symptoms and improve daily functioning through:
• Physical exercise
• Breathing techniques to improve oxygen use
• Nutritional counseling
• Emotional counseling and support
• Education about your condition

When symptoms suddenly get worse
A sudden worsening of symptoms, called an acute exacerbation, may require increased supplemental oxygen. Some cases require mechanical ventilation in the hospital. Treatments may include antibiotics, corticosteroids or other medicines.

Lung transplant
A lung transplant may be an option for some people. It can improve quality of life and extend survival. However, it carries risks such as infection and organ rejection. Lifelong medicines are required after the procedure. Your healthcare team can discuss whether a transplant is a suitable option for your condition.