“Pulmonary” means lung, and “fibrosis” means scarring. Pulmonary fibrosis, or PF, is when something scars and stiffens your lung tissue. It can make it hard for your lungs to work the right way.
Your doctor will call it idiopathic pulmonary fibrosis, or IPF, if they’re not sure why it happened.
Your symptoms, and how severe they are, depend on what’s causing your pulmonary fibrosis. It’s different for everyone. The two most common symptoms are:
Shortness of breath. At first, you might notice you get short of breath more easily when you exercise. As the disease gets worse, you may have a hard time breathing even if you’re not doing anything hard.
Dry cough. You may have coughing fits that you can’t seem to stop. It typically gets worse over time.
Other signs and symptoms may include:
It can be hard to figure out the reason for your pulmonary fibrosis. The possible causes scientists do know about include:
- Drugs for abnormal heart rhythms (such as amiodarone and others)
- Drugs for inflammation (such as Rituxan or Azulfidine)
- Chemotherapy drugs for cancer (such as methotrexate and cyclophosphamide)
- Antibiotics for urinary tract infections (such as nitrofurantoin and others)
Autoimmune diseases. Your doctor might call these rheumatologic or connective tissue diseases. Autoimmune conditions that may lead to pulmonary fibrosis include:
Environmental causes. These might be natural substances like mold spores, bacteria, or even dust from animal poop, especially birds. Doctors call this type of lung inflammation and scarring hypersensitivity pneumonitis, or HP. It’s not clear why some lungs form HP and others don’t.
You’re more likely to breathe in substances like dust from asbestos, silica, coal, beryllium, and hard metals in certain work settings. This can lead to inflammation and scarring called pneumoconiosis.
Tobacco smoke is another possible environmental cause of lung fibrosis.
The doctor will ask about your symptoms, health history, and all medications you take. There are a few initial signs that might point to PF:
A consistent crackling sound. Your doctor may hear this through the stethoscope when you breathe. (This also could be a sign of a different lung problem.)
Not enough oxygen in your blood. Your doctor can easily check this with a small, painless clip on your finger.
Your lungs don’t work as well as they should. Scarred and stiff lung tissue is less stretchy and can shrink the possible air space inside your lungs. Your doctor can check both the strength and space of your lungs with a machine called a spirometer.
Imaging. This may include a chest X-ray, but often you’ll need a more detailed picture from a special high-resolution CT scan (HRCT). Your doctor might also order an echocardiogram to check how well your heart works, which may show signs of problems because of PF.
Biopsy. This is when the doctor takes a small sample of tissue from your lungs to look at under a microscope.
The goal of treatment is to improve symptoms and quality of life, and keep your condition from getting worse. They include:
Medication. Drugs like pirfenidone (Esbriet) and nintedanib (Ofev) may help slow lung damage. Your doctor might also suggest medication for gastroesophageal reflux disease (GERD), which happens often in people with pulmonary fibrosis.
Oxygen therapy. This is when you breathe in oxygen through nose tubes or a face mask. It won’t stop lung damage, but it can:
- Help your breathing
- Ease problems from low blood oxygen
- Boost energy
- Help you sleep better
Breathing exercises. These can help your lungs work better and for longer periods of time.
Lung transplant. Your doctor might suggest surgery if your lungs are really damaged.