Pulmonary fibrosis is a rare chronic lung condition that causes lung tissue to become scarred and stiff. When you have pulmonary fibrosis, scar tissue makes it hard for oxygen to move into the lungs. This reduces the amount of nourishing oxygen that gets into your bloodstream. The scarring, or fibrosis, cannot be reversed. Because of this, pulmonary fibrosis may get worse over time, but with a doctor's help, patients can breathe easier and slow disease progression.
Pulmonary fibrosis is one of more than 200 lung conditions that make up a larger category of conditions called interstitial lung disease (ILD). Interstitial lung diseases cause the tiny air sacs in the lungs to have inflammation and scarring. Because many of these lung conditions have similar symptoms, diagnosing pulmonary fibrosis can be challenging.
Pulmonary Fibrosis Program at Temple
Pulmonary fibrosis can be difficult to diagnose because symptoms are similar to that of other conditions. Because of this, it’s important to work with lung specialists who have the experience to recognize the condition and treat it effectively. While there is no cure for pulmonary fibrosis, advanced treatments can help to reduce debilitating symptoms and slow down the progression of the disease.
Temple’s Pulmonary Fibrosis Program is a leader in the field of lung disease treatments and offers many options, including lifestyle modifications, medications, supplemental support and lung transplant. For those with all forms of pulmonary fibrosis, an early and accurate diagnosis can make all the difference in the quality of life.
Causes of Pulmonary Fibrosis
There are generally four different causes of pulmonary fibrosis. These are:
Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic pulmonary fibrosis means that the cause of the pulmonary fibrosis is not known, and is the most common diagnosis patients receive.
Pulmonary Fibrosis From Diseases
Pulmonary Fibrosis From Environmental or Chemical Exposure
You can develop pulmonary fibrosis as the result of breathing in toxins such as asbestos. You can also develop pulmonary fibrosis from exposure to bird and other animal droppings and certain medications or treatments, such as radiation treatment for cancer.
Familial Pulmonary Fibrosis
When certain types of interstitial lung diseases occur in two or more people in your family, it’s called familial pulmonary fibrosis. This form of pulmonary fibrosis is rare and not well understood, but researchers suspect genetics play a role.
Determining Pulmonary Fibrosis Severity
Doctors use a combination of lung function tests, your need for supplemental oxygen and the effect your symptoms have on your everyday life to determine the severity of your pulmonary fibrosis. Spirometry is the most common form of lung function test. It measures:
- Forced Vital Capacity (FVC) - How much oxygen you can breathe in and out of your lungs.
- Forced Expiratory Volume (FEV) - How fast you can exhale within a certain amount of time.
Using this information, doctors identify the severity of your pulmonary fibrosis. This helps in deciding what type of treatment you may need. In general, the severity ranges from mild to very severe.
Risk Factors for Pulmonary Fibrosis
A risk factor is something that makes you more likely to get a condition. Risk factors for pulmonary fibrosis include:
- Age over 50 years
- Cancer treatments, including radiation treatment and certain medications
- Working in environments such as farming, mining or construction
The most common symptom of pulmonary fibrosis is shortness of breath or breathlessness with activity. Other symptoms include:
- Dry cough
- Muscle and joint aching
- Weight loss
- Widening of the tips of fingers and toes (clubbing)
If you have one or a combination of these symptoms, ask your doctor if it’s time to see a lung specialist.
Pulmonary fibrosis can be difficult to distinguish from other lung diseases. That’s why the diagnosis of pulmonary fibrosis is based on a number of factors. Your lung specialist will talk to you about your medical history, your symptoms, and any family history you may have of lung disease. You will also have a number of tests that may include:
- Biopsy or sample of your lung tissue
- Blood tests to see how your liver and kidneys are functioning
- Chest X-ray and other imaging exams
- Lung function tests, including spirometry
You may also have tests to rule out other conditions, such as tuberculosis.
Learn more about specific tests used to diagnose pulmonary fibrosis >
Pulmonary fibrosis typically gets worse over time. While the scarring is not reversible, every person’s experience of pulmonary fibrosis is different. Depending on the type of pulmonary fibrosis you have, treatments may slow the progression of the disease and can lead to improvements in quality of life. Early and accurate diagnosis, following your treatment plan, and close communication with your pulmonary fibrosis team helps give you the best possible outcome