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. When no known cause is identified, the disease is called idiopathic pulmonary fibrosis (IPF).
- IPF is a type of interstitial lung disease (ILD) meaning it affects the interstitium, which refers to the tissue around the air sacs of lung.
- There is no cure for IPF.
Patient and Clinician Engagement in Treating Pulmonary Fibrosis Webinar
Understanding where the patient is within their progression of pulmonary fibrosis, as well as any emotional barriers and stigmas, allows clinicians to treat the person rather than just the disease. Meeting patients where they are empowers them to speak directly about the concerns and issues they currently face. At the same time, chest medicine clinicians who have a more objective picture of their patients’ progression can tailor treatments and better create an atmosphere where patients are willing to engage and feel motivated to take charge of the management of pulmonary fibrosis.
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. Once present, this scarring 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 breathe.
How Pulmonary Fibrosis Affects Your Body
Normal lungs have no scarring and this means oxygen can pass easily from the environment into the air sacs of the lungs and easily pass into the bloodstream. This allows for oxygenation of the blood. In pulmonary fibrosis, the lungs lack elasticity and are stiff due to scarring or fibrosis. This scarring makes it harder for oxygen to pass through the alveoli (air sacs) and reduces the amount of oxygen that can be passed into the bloodstream. The fibrosis stiffens the lungs, reducing their size and capacity. This reduction in lung capacity is reflected in breathing tests, also known as pulmonary function tests, which may be used to diagnose or monitor pulmonary fibrosis progression. As the scarring or fibrosis progresses it may become harder to breathe 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, making it harder for oxygen to diffuse into the bloodstream. In most cases of pulmonary fibrosis, the cause is not found. In other cases, the causes of pulmonary fibrosis may 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 examine you and listen to your lungs with a stethoscope for abnormal breath sounds. Your provider may also order the following tests to assess for the presence and impact of fibrosis:
- Pulmonary Function Testing: This test involves breathing quickly and forcefully through a tube that is connected to a machine. It 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 is most appropriate.
- Chest X-ray: Can show scarring of the lungs that may indicate pulmonary fibrosis.
Computerized tomography (CT): This is a test in which many x-ray images are taken 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.
- Blood tests: These tests are used to evaluate blood markers that may suggest an underlying autoimmune etiology for pulmonary fibrosis, including, for example, rheumatoid arthritis, lupus and scleroderma
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 scarring.
The following are some of the medications your healthcare provider may choose, depending on testing and what may be beneficial in each individual case:
- 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 yet.
- Cyclophosphamide (Cytoxan): This is a chemotherapy drug that can also suppress inflammation.
Mycophenolate mofetil (Cellcept): This drug and also azathioprine are anti-inflammatories that have been used in treating autoimmune diseases, including rheumatoid arthritis.
- Nintedanib (Ofev): 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 decline in lung function, as measured by pulmonary function tests.
- Pirfenidone (Esbriet Pirfenex): 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. It is approved for the treatment of idiopathic pulmonary fibrosis in the United States, Canada, the EU, and Asia.
- Proton Pump Inhibitors: Gastroesophageal reflux has been shown to worsen pulmonary fibrosis. Therefore, physicians will make pharmacologic and nonpharmacologic recommendations to treat reflux.
- N-acetylcyseine (NAC): NAC is an antioxidant but has not been found to have beneficial impact in pulmonary fibrosis.
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 with exertion or all the time to help increase the level of oxygen in the bloodstream, which may be reduced by the pulmonary fibrosis. When used as directed, oxygen can improve shortness of breath and help keep patients active.
- Pulmonary Rehabilitation: Pulmonary Rehabilitation is a formal exercise program for improving the strength of the muscles that are involved in breathing. In addition to exercise, a rehabilitation program may also include stress management, anxiety counseling and other educational components. A healthcare provider must prescribe pulmonary rehabilitation and specify the particular level of activity appropriate for the patient.
- 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 out 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?
Print and complete our ILD/IPF patient questionnaire to help you initiate a conversation with your doctor about ILD/IPF
Marie M. Budev, DO, FCCP
Samantha D’Annunzio, MD
Sheetal Gandotra, MD
Date Last Reviewed