What is pulmonary fibrosis?
Pulmonary fibrosis (PF) is scarring of the lung tissue. It is a type of interstitial lung disease, which includes more than 200 diseases. Interstitial lung tissue (interstitium) surrounds and supports the air sacs. As scarring develops, the tissue becomes thick and stiff. This makes it hard for the lungs to work and get oxygen into your blood. The result is shortness of breath, especially with exertion. As the disease progresses, shortness of breath and other symptoms worsen.
In most cases, there is no known cause for the scarring. The name for this is idiopathic pulmonary fibrosis. Sometimes, doctors can link the scarring to an underlying cause. This can include drugs, radiation, autoimmune diseases, and inhaled irritants. Certain occupations can increase the risk of PF due to exposure to dust, fibers or fumes. Smoking is also a risk factor for the disease.
Along with shortness of breath, pulmonary fibrosis causes dry cough, fatigue, and weight loss. Progression of these symptoms is rather unpredictable. Some people experience mild disease that remains stable for a long time. Others will have severe disease that progresses quickly. Still others will be stable and then quickly decline for unknown reasons.
Currently, there is no cure for PF. Medications can help manage the symptoms, but they will not reverse the scarring. In some cases, doctors recommend lung transplant.
Acute exacerbations—or flares—can happen with PF and become life threatening. Seek immediate medical care for serious symptoms, including severe shortness of breath, inability to catch your breath, chest pain or discomfort, or bluish skin, lips or nails.
What are the symptoms of pulmonary fibrosis?
Pulmonary fibrosis is a progressive disease, but it is also unpredictable. The severity of symptoms and course of the disease can vary from person to person. The disease can remain stable for years with mild to moderate symptoms. Alternatively, it can cause severe symptoms that progress very quickly. It can also appear stable, but suddenly worsen without an apparent reason.
Common symptoms of pulmonary fibrosis
The most common pulmonary fibrosis symptoms are:
Clubbing, which is widening and rounding of the fingertips and toes
Dry, persistent cough
Fatigue or weakness
Joint and muscle aches
Shortness of breath, especially with activity or exertion
Serious symptoms that might indicate a life-threatening condition
In some cases, people with PF can experience severe flares. These flares are potentially life threatening. Seek immediate medical care (call 911) if you, or someone you are with, have any of these life-threatening symptoms including:
Chest pain or severe discomfort
Cyanosis, which is a bluish tint to the skin, lips or nails due to lack of oxygen
Inability to catch your breath or hungering for air
Severe shortness of breath
Severe flares require hospitalization. Some people will need a ventilator to help them breathe. When the flare is this severe, the mortality rate is high. If you have PF, talk with your doctor and family about your wishes should you end up in this situation. Because the disease is unpredictable, you should complete an advanced directive to spell out your wishes as soon as possible after diagnosis. It will tell your doctor and family what to do to honor your wishes.
What causes pulmonary fibrosis?
In most cases, doctors are not able to identify a specific cause of PF. When the cause is unknown, doctors call it idiopathic. When doctors can find a cause, it may include:
Chest radiation treatments
Environmental exposure to mold, bacteria, or bird or animal droppings or proteins
Occupational exposure to fibers, fumes or dust, including asbestos, coal, grain, hard metal, and silica
What are the risk factors for pulmonary fibrosis?
Working in an occupation that exposes you to potential triggers can increase the risk of PF. Examples include construction workers, metal workers, farmers, and hairstylists. Several other factors increase the risk of developing PF including:
Age 50 to 70 years. Most people are older than 60 at diagnosis.
Genetics. Some forms of PF run in families, can be inherited, or are linked to specific genes.
Smoking or history of smoking
Reducing your risk of pulmonary fibrosis
Reducing the risk of a disease often relies on changing risk factors you can control. Unfortunately, most cases of PF are not preventable because the cause is unknown. If you have risk factors, you may be able to reduce your risk by:
Following all safety precautions if your work exposes you to potential triggers
Stopping smoking or never starting
Seeing your doctor for regular medical care can help catch problems early. If you notice persistent lung symptoms between checkups, contact your doctor for a visit.
How is pulmonary fibrosis treated?
The lung tissue scarring and damage in PF is irreversible. There is no way to cure the disease today. But there are treatments available to help control symptoms and improve your quality of life. If your doctor knows there is an underlying condition, treating the condition can also help. Pulmonary fibrosis treatment options include:
Medications to manage the symptoms of PF. Anti-inflammatory medicines, such as corticosteroids, are useful in some forms of the disease. There are also two drugs with FDA approval to treat idiopathic pulmonary fibrosis—nintedanib (Ofev) and pirfenidone (Esbriet). These drugs can’t reverse the scarring, but they can slow the progression of the disease and preserve lung function.
Oxygen therapy to keep your oxygen levels from falling below 88%
Pulmonary rehabilitation to teach you how to function with less shortness of breath. This may include learning breathing exercises, stress management, nutrition counseling, and exercise training.
Transplantation of one or both lungs to prolong your life. This surgery may be an option for eligible patients. However, very few lungs are available each year for transplantation. Only about 1,000 PF patients receive a lung transplant each year.
What are the potential complications of pulmonary fibrosis?
It can be difficult for doctors to offer a confident prognosis with PF. Everyone experiences the disease differently and doctors can’t predict how it will progress. There are also no formal pulmonary fibrosis stages. In general, doctors describe the disease as mild, moderate, severe, or very severe. They use your symptoms, lung function tests, and other information to gauge the severity. Complications can develop as the disease progresses.
Potential pulmonary fibrosis complications include: