Learn about Pulmonary Fibrosis
Pulmonary fibrosis is a rare but serious lung disease that causes the lung tissue to become thickened, scarred and stiff. When lung scarring occurs, it is called pulmonary fibrosis.
- In most cases doctors do not know what causes pulmonary fibrosis and is called idiopathic pulmonary fibrosis (IPF) when no known cause is identified
- Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease (ILD) meaning it affects the interstitium or the air sacs around the lung.
- There is no cure for IPF.
What is idiopathic pulmonary fibrosis?
Pulmonary fibrosis is irreversible scarring of the lung. When no reason or cause for the scarring is found it is termed idiopathic. Most cases of fibrosis have no cause identified. The scarring or fibrosis of the lung causes the lungs to be stiff and affects how much air the lungs can take in during a breath. This scarring once present cannot be reversed or repaired. As the lungs continue to get more scarred or fibrotic, patients become more short of breath and may need oxygen to help them breath.
How Pulmonary Fibrosis Affects Your Body
Normal lungs have no scarring and this means oxygen can pass easily from the lungs into and around the air sacs which make up the lungs and easily pass into the bloodstream. This allows for oxygenation of the blood. In pulmonary fibrosis, the lungs no longer have elasticity and are stiff due to scarring or fibrosis. This scarring makes it harder for oxygen to pass around the alveoli or air sacs and reduces the amount of oxygen that can be passed into the bloodstream. The lungs will be stiffer due to the fibrosis and this will also reduce the size of the lungs and will impact breathing tests or pulmonary function tests that may be used to diagnose or monitor pulmonary fibrosis progression. As the scarring or fibrosis progresses it may become harder to breath and some patients may need oxygen to help them breathe.
How Serious is Pulmonary Fibrosis?
The scarring or fibrosis that happens with pulmonary fibrosis cannot be reversed or repaired. Unfortunately, there is no known cure for idiopathic pulmonary fibrosis. Unlike other common chronic diseases such as diabetes or high blood pressure, pulmonary fibrosis is rare. Pulmonary fibrosis affects up to an estimated 132,000 people in the United States with almost 50,000 newly diagnosed cases per year. The symptoms of pulmonary fibrosis are similar to those of other lung diseases including asthma, and chronic obstructive lung disease (COPD) . In fact, since the symptoms are so similar up to 50% of cases are misdiagnosed on initial presentation. Establishing the correct diagnosis of pulmonary fibrosis is important so that a doctor may be able to help care and treat breathlessness which is a common symptom of pulmonary fibrosis.
- Pulmonary fibrosis can occur at any age but usually presents between the ages of 50 and 70.
- Pulmonary fibrosis occurs more often in men than women
- Pulmonary fibrosis occurs irrespective of race, ethnicity or social status.
Pulmonary Fibrosis Symptoms, Causes, and Risk Factors
The severity of symptoms associated with pulmonary fibrosis varies from person to person. Some individuals will get very sick quickly when the disease is severe. Other people will have mild symptoms for years with no decline or worsening with mild disease. The natural course of pulmonary fibrosis also varies from person to person but the overall course for pulmonary fibrosis can be unpredictable. People who have been stable for years may have a sudden unexplained progression of their disease.
Signs and symptoms of pulmonary fibrosis include:
- Shortness of breath
- A dry cough
- Unintentional weight loss
- Muscle and joint aching
What Causes Pulmonary Fibrosis?
Pulmonary fibrosis happens when the lung tissue becomes thickened and scarred around and between the lung sacs or lung alveoli. This makes the lungs stiff and harder for oxygen to diffuse or get into the bloodstream. In most cases of pulmonary fibrosis the cause is not found. In other cases, the scarring of the lung can include some of the following:
- Occupational factors including silica, asbestos , grain dust
- Environmental factors including bird and animal droppings
- Radiation treatments
- Medications including chemotherapy drugs, heart medications including amiodarone and some antibiotics including nitrofurantoin and sulfasalazine
- Other medical illnesses including systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis and scleroderma.
What Are Risk Factors?
- Age: Pulmonary fibrosis is more likely in 50-70 year olds than in younger patients
- Smoking: More smokers or former smokers develop pulmonary fibrosis than non-smokers.
- Occupation: Certain occupations including mining, farming or construction may increase the risk for pulmonary fibrosis.
- Cancer treatment: Radiation treatments and certain chemotherapy can increase the risk for pulmonary fibrosis.
- Genetic Factors: Some types of pulmonary fibrosis may occur more often in families and a genetic cause has been suggested.
When to See Your Doctor
If you have a family history of pulmonary fibrosis or have some of the risk factors and symptoms of pulmonary fibrosis, you should consult your healthcare provider.
Diagnosing Pulmonary Fibrosis
When you see your healthcare provider, your provider will exam you and listen to your lungs with a stethoscope for abnormal breath sounds. Your provider may also order the following tests to assess the amount of fibrosis present:
- Pulmonary Function Testing: This test involves breathing quickly and forcefully through a tube that is connected to a machine and measures the amount of air in the lungs and how much air can move in and out of the lungs.
- Oximetry: This test uses a small device that is placed on the tip of the finger and measures the oxygen saturation in the blood.
- Tissue Biopsy: Sometimes doctors need a small amount of lung tissue to definitively diagnose pulmonary fibrosis. Tissue biopsies can be obtained in several ways either by an outpatient procedure called a bronchoscopy or using a more invasive procedure to obtain tissue called a surgical biopsy. Your healthcare provider will determine if a biopsy is necessary and will determine if a surgical or bronchoscopic biopsy.
- Chest X-ray: Can show the scarring of the lungs that may indicate pulmonary fibrosis. This test is often used on subsequent visits by the provider to follow the progression of the disease.
- Computerized tomography (CT): This is a test in which many x-ray images are taken from different angles to provide more detailed images of the lung allowing healthcare providers to determine the extent of lung damage or scarring caused by pulmonary fibrosis.
- Echocardiogram: This is an ultrasound or sonogram of the heart and allows doctors to determine if the pressures in the heart have been impacted by the scarring of the lungs.
Treatment of Pulmonary Fibrosis
There is no current treatment that has proved effective in stopping the progression of pulmonary fibrosis. Some treatments may help improve the symptoms for a while or slow down the progression of the disease.
Some of the medications your healthcare provider may choose depending on testing and what may be beneficial in each individual case and may include:
- Corticosteroids (prednisone): These drugs are used to suppress the immune system and decrease inflammation which may have some impact on lung tissue that is not fully scarred.
- Cyclophosphamide (CytoxanÒ) : This is a drug used for chemotherapy that can also suppress inflammation.
- Mycophenolate mofetil (CellceptÒ): This drug and also azathioprine (ImuranÒ) are anti-inflammatories that have been used in treating autoimmune diseases including rheumatoid arthritis.
- N-acetylcyseine (NAC): NAC is an antioxidant but has not been found to have beneficial impact in pulmonary fibrosis.
- Nintedanib (OfevÒ) : Nintedanib is a antifibrotic drug that is approved to treat idiopathic pulmonary fibrosis in the United States and has been shown in clinical trials to slow down the drop in pulmonary function tests.
- Pirfenidone (EsbrietÒ PirfenexÒ) Pirfenidone is another anti-fibrotic and anti-inflammatory that has been shown in clinical trials to slow down the progression of mild and moderate pulmonary fibrosis and is approved for the treatment of idiopathic pulmonary fibrosis in the United States, Canada, the EU, and Asia.
Other than medications, health-care providers may suggest the following treatments for the management of the symptoms of pulmonary fibrosis:
- Supplemental Oxygen Therapy: Healthcare providers may prescribe oxygen to be worn either on exertion or all the time to help increase the level of oxygen in the bloodstream which may be reduced by the pulmonary fibrosis. Oxygen when used as directed can prevent shortness of breath and can keep patients active.
- Pulmonary Rehabilitation: Pulmonary Rehabilitation is a formal exercise program for improving the strength of the muscles that are involved in breathing. A rehabilitation program also may include besides exercise training but also stress, anxiety counseling and other educational components. A healthcare provider must prescribe pulmonary rehabilitation and specify the particular level of activity.
- Lung Transplantation: Pulmonary fibrosis is the leading cause for lung transplantation in the United States today. Lung transplant improves both survival and quality of life in patients with pulmonary fibrosis. Although both the surgical technique and medical management of patients has improved, lung transplant is not without risks and should be discussed in detail with a healthcare provider to understand the risks involved.
Living with Pulmonary Fibrosis
Your healthcare team will help you manage your disease and symptoms but living with pulmonary fibrosis means a patient must speak for themselves, be prepared for physician visits, letting friends and family help, and contacting support groups (i.e. including the pulmonary fibrosis Foundation web) site to find out how others manage their disease.
What To Expect
Each patient with pulmonary fibrosis may have a different course and symptoms as the disease progresses. Some patients may have periods where their disease remains stable with no change in symptoms. It is important that the healthcare team is kept informed of any changes in a patient’s symptoms.
Managing the Disease
Managing all the different aspects of the disease can be difficult it is important to keep the healthcare team updated and all medical records organized. In addition, it is important to maintain a healthy lifestyle including tobacco cessation, regular exercise (including pulmonary rehabilitation), and following a healthy diet in addition to remaining compliant with medications and oxygen therapy.
It is important to seek the help from local and web based support groups. This can help to find solutions or to find our how others manage their disease and symptoms. The Pulmonary Fibrosis Foundation has a detailed website that can be a valuable resource for information and for finding local support groups and meetings.
Questions to Ask Your Doctor
Your healthcare team is important in the management of your disease and your symptoms. Providers are there to provide information to help, some questions to ask may include:
- Who should I contact if I have problems with my pulmonary fibrosis?
- When should I start therapy or switch therapies for my fibrosis?
- Are there clinical trials for me to participate in?
- When should I discuss end of life or hospice?
Marie M. Budev, DO, FCCP
Date Last Reviewed