Pulmonary fibrosis is a lung disease characterized by scarring and thickening of the lung tissue. Over time, the scar tissue replaces healthy lung tissue, making it harder for the lungs to function properly and leading to symptoms such as shortness of breath, coughing, fatigue, and chest pain.

There are several different types of pulmonary fibrosis, and the cause of the disease is often unknown. Some types of pulmonary fibrosis may be caused by exposure to certain environmental factors such as silica dust, asbestos, or animal droppings. Other types may be caused by autoimmune diseases or genetic factors.

Treatment for pulmonary fibrosis typically focuses on slowing the progression of the disease and managing symptoms. This may involve the use of medications to reduce inflammation or suppress the immune system, oxygen therapy to improve breathing, pulmonary rehabilitation to improve lung function and quality of life, and in some cases, lung transplantation.

Pulmonary fibrosis is a chronic and progressive disease, and there is currently no cure. However, with proper treatment and management, many people with pulmonary fibrosis are able to maintain a good quality of life and continue to participate in daily activities for many years.

Pulmonary fibrosis scars and thickens the tissue around and between the air sacs (alveoli) in your lungs. This makes it more difficult for oxygen to pass into your bloodstream. The damage can be caused by many different factors — including long-term exposure to certain toxins, certain medical conditions, radiation therapy and some medications.

Occupational and environmental factors

Long-term exposure to a number of toxins and pollutants can damage your lungs. These include:

• Silica dust
• Asbestos fibers
• Hard metal dusts
• Coal dust
• Grain dust
• Bird and animal droppings

Radiation treatments

Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment. The severity of the damage may depend 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

Medications

Many drugs can damage your lungs, especially medications such as:

• Chemotherapy drugs. Drugs designed to kill cancer cells, such as methotrexate (Trexall, Otrexup, others) and cyclophosphamide, can also damage lung tissue.
• Heart medications. Some drugs used to treat irregular heartbeats, such as amiodarone (Cordarone, Nexterone, Pacerone), may harm lung tissue.
• Some antibiotics. Antibiotics such as nitrofurantoin (Macrobid, Macrodantin, others) or ethambutol can cause lung damage.
• Anti-inflammatory drugs. Certain anti-inflammatory drugs such as rituximab (Rituxan) or sulfasalazine (Azulfidine) can cause lung damage.

Medical conditions

Lung damage can also result from a number of conditions, including:

• Dermatomyositis
• Polymyositis
• Mixed connective tissue disease
• Systemic lupus erythematosus
• Rheumatoid arthritis
• Sarcoidosis
• Scleroderma
• Pneumonia

Many substances and conditions can lead to pulmonary fibrosis. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.

Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. Also, some forms of idiopathic pulmonary fibrosis run in families, and heredity may play a role in idiopathic pulmonary fibrosis.

Many people with idiopathic pulmonary fibrosis may also have gastroesophageal reflux disease (GERD) — a condition that occurs when acid from your stomach flows back into your esophagus. Ongoing research is evaluating if GERD may be a risk factor for idiopathic pulmonary fibrosis, or if GERD may lead to a more rapid progression of the condition. However, more research is needed to determine the association between idiopathic pulmonary fibrosis and GERD.

The lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in stopping progression of the disease. Some treatments may improve symptoms temporarily or slow the disease's progression. Others may help improve quality of life. Doctors will evaluate the severity of your condition to determine the most appropriate treatment for your condition.

Medications

Your doctor may recommend newer medications, including pirfenidone (Esbriet) and nintedanib (Ofev). These medications may help slow the progression of idiopathic pulmonary fibrosis. Both medications have been approved by the Food and Drug Administration (FDA). Additional medications and new formulations of these medications are being developed but have not yet been FDA approved.

Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include rash, nausea and diarrhea.

Researchers continue to study medications to treat pulmonary fibrosis.

Doctors may recommend anti-acid medications to treat gastroesophageal reflux disease (GERD), a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.

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 may receive oxygen when you sleep or exercise, although some people may use it all the time. Some people carry a canister of oxygen, making them more mobile.

Pulmonary rehabilitation

Pulmonary rehabilitation can help you manage your symptoms and improve your daily functioning. Pulmonary rehabilitation programs focus on:

• Physical exercise to improve your endurance
• Breathing techniques that may improve lung efficiency
• Nutritional counseling
• Counseling and support
• Education about your condition

Lung transplant

Lung transplantation may be an option for people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. However, a lung transplant can involve complications such as rejection and infection. Your doctor may discuss with you if a lung transplant may be appropriate for your condition.