Pulmonary fibrosis occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.
The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can’t pinpoint what’s causing the problem. When a cause can’t be found, the condition is termed idiopathic pulmonary fibrosis.
The lung damage caused by pulmonary fibrosis can’t be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate
Symptoms
Signs and symptoms of pulmonary fibrosis include:
Shortness of breath (dyspnea)
A dry cough
Fatigue
Unexplained weight loss
Aching muscles and joints
The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have more-moderate symptoms that worsen over months or years.
Causes
1. Occupational and environmental factors
Long-term exposure to a number of toxins and pollutants can damage your lungs. These may include:
Silica dust
Asbestos fibers
Grain dust
Bird and animal droppings
2. Radiation treatments
Some people who receive radiation therapy for lung or chest cancer show signs of lung damage months or sometimes years after the initial treatment. The severity of the damage depends on:
How much of the lung was exposed to radiation
The total amount of radiation administered
Whether chemotherapy also was used
The presence of underlying lung disease
3. Medications
Many drugs can damage your lungs, especially:
Chemotherapy drugs. Drugs designed to kill cancer cells, such as methotrexate (Trexall) and cyclophosphamide (Cytoxan), can also damage lung tissue.
Heart medications. Some drugs used to treat irregular heartbeats, such as amiodarone (Cordarone, Nexterone, Pacerone) or propranolol (Inderol, Inderide, Innopran) may harm lung tissue.
Some antibiotics. Nitrofurantoin (Macrobid, Macrodantin, others) and sulfasalazine (Azulfidine) can cause lung damage.
4. Medical conditions
Lung damage can also result from:
Tuberculosis
Pneumonia
Systemic lupus erythematosus
Rheumatoid arthritis
Sarcoidosis
Scleroderma
5. Idiopathic pulmonary fibrosis: When the cause isn’t known
The list of substances and conditions that can lead to pulmonary fibrosis is long. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.
Complications
Complications of pulmonary fibrosis may include:
High blood pressure in your lungs (pulmonary hypertension). Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. It begins when the smallest arteries and capillaries are compressed by scar tissue, causing increased resistance to blood flow in your lungs. This in turn raises pressure within the pulmonary arteries. Pulmonary hypertension is a serious illness that becomes progressively worse and may eventually prove fatal.
Right-sided heart failure (cor pulmonale). This serious condition occurs when your heart’s lower right chamber (ventricle) has to pump harder than usual to move blood through partially blocked pulmonary arteries.
Respiratory failure. This is often the last stage of chronic lung disease. It occurs when blood-oxygen levels fall dangerously low.
Lung cancer. Long-standing pulmonary fibrosis also increases your risk of developing lung cancer
Treatments and Drugs
The lung scarring that occurs in pulmonary fibrosis can’t be reversed, and no current treatment has proved effective in stopping the ultimate progression of the disease. Some treatments, though, may improve symptoms temporarily or slow the disease’s progress. Others help improve quality of life.
Medications
Many people diagnosed with pulmonary fibrosis are initially treated with a corticosteroid (prednisone), sometimes in combination with other drugs that suppress the immune system — such as methotrexate or cyclosporine. None of these combinations has proved very effective over the long run. Adding N-acetylcysteine, a derivative of a natural amino acid, to prednisone may slow the disease in some people.
1. Oxygen therapy
Using oxygen can’t stop lung damage, but it can:
Make breathing and exercise easier
Prevent or lessen complications from low blood-oxygen levels
Reduce blood pressure in the right side of your heart
Improve your sleep and sense of well-being
You’re most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.
2. Pulmonary rehabilitation
The aim of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning, but also to help people with pulmonary fibrosis live full, satisfying lives. To that end, pulmonary rehabilitation programs focus on:
Physical exercise, to improve your endurance
Breathing techniques that improve lung efficiency
Emotional support
Nutritional counseling
3. Surgery
Lung transplantation may be an option of last resort for younger people with severe pulmonary fibrosis who haven’t benefited from other treatment options.
Prevention
Being actively involved in your own treatment and staying as healthy as possible are essential to living with pulmonary fibrosis. For that reason, it’s important to:
Stop smoking. If you have lung disease, the best thing you can do for yourself is to stop smoking. Talk to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit. And because secondhand smoke can be just as harmful to your lungs, don’t allow other people to smoke around you.
Eat well. People with lung disease may lose weight both because it’s uncomfortable to eat and because of the extra energy it takes to breathe. Yet a nutritionally rich diet that contains adequate calories is essential. A dietitian can give you further guidelines for healthy eating.
Get vaccinated. Respiratory infections can worsen symptoms of pulmonary fibrosis. Make sure you receive the pneumonia vaccine and an annual flu shot.