Pulmonary fibrosis happens when the lung tissue becomes scarred. This scarred tissue becomes thicker and stiffer. As this happens, your lungs cannot work as well and have difficulty getting oxygen into the blood. Lack of oxygen makes it difficult for the body to work well, and causes shortness of breath.
When the cause for pulmonary fibrosis is not found, the condition is known as idiopathic pulmonary fibrosis (IPF).
People with pulmonary fibrosis may have different symptoms. The symptoms commonly happen over time and slowly get worse. Some patients may notice symptoms that get worse very quickly. Symptoms may also differ depending on the cause of the condition.
Symptoms of pulmonary fibrosis include:
- Shortness of breath, particularly during exercise
- Dry cough
- Fast, shallow breathing
- Unexplained weight loss
- Achy joints and muscles
Causes and Risk Factors
Pulmonary fibrosis is related to many conditions, but in most cases, the cause is never found. When this is the case, the condition is known as idiopathic pulmonary fibrosis. Research is looking into what might cause idiopathic pulmonary fibrosis, including viruses and tobacco smoke. Some types of the disease appear to run in families.
These factors may increase risk of pulmonary fibrosis:
- Work-related and environmental toxins.
- Radiation treatments
- Medications, including chemotherapy drugs
- Autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis
Most of the people who get the disease are middle-aged and older adults, people who smoke, and those who work with chemicals that damage the lungs.
Diagnosing pulmonary fibrosis usually begins with a physical exam. Your doctor will ask if you smoke, whether you have lived or worked with materials that could harm your lungs, drug use and other health conditions.
Imaging diagnostic tests may include chest X-rays and CT scans. These tests can show what is going on inside the lungs including the amount of damage, if fluid has begun to build up in the chest or lungs and if the lungs filling up with as much air as possible.
Other tests may include pulmonary function tests and bronchoscopy to find out how much damage has been done and how well the lungs are working.
If fluid has begun to build up in the chest or lungs, the doctor may take a sample of the fluid for testing to rule out cancer.
There is no way to repair scarring that has already happened to the lungs. Available treatments for pulmonary fibrosis aim to stop more damage and easy symptoms. The experts at the Advanced Lung Disease Program can help you determine the best treatment plan.
Anti-inflammatory medicines, such as corticosteroids, or other medicines that suppress the immune system may help prevent more scarring and add to the lifespan of people whose condition is related to an autoimmune disease. But, they may not work for every patient with pulmonary fibrosis.
Oxygen therapy may be used if the amount of oxygen in the blood is low. This may help with shortness of breath and let you remain active longer. Many doctors will recommend pulmonary rehabilitation, which gives information and counseling to help manage symptoms.
It also helps to stop smoking.
For some, a lung transplant may be the best option when other treatments have not worked. Anyone younger than 65 with no other health problems might be given a lung transplant.