Severity of Pulmonary Fibrosis

Pulmonary fibrosis is often a progressive disease that gets worse over time. No one can predict how fast your PF will progress. In some people, PF progresses very quickly while others live with the disease for years. 

How Severe is my Pulmonary Fibrosis?

When someone is diagnosed with pulmonary fibrosis, it is normal to wonder how advanced the disease is. There is no formal staging system for pulmonary fibrosis. Physicians use different factors, like the ones listed below, to describe the disease as mild, moderate, severe or very severe:

• Symptoms: Discussing symptoms with your healthcare provider is key to determining the severity of your PF. You might be asked about how frequently you become breathless or how often you cough. Worsening breathlessness and cough don't always mean the disease has progressed but knowing your symptoms and what is typical for you helps your healthcare provider better understand your disease.
• Pulmonary Function Tests (PFTs) give your healthcare provider important information about the amount of air the lungs can hold and how forcefully you can empty air from the lungs. PFTs reveal your  forced vital capacity (FVC), which is the amount of air that is exhaled starting from a maximal inhalation, how much air your lungs can hold and how easily it is for gases to move between your blood and lungs These percentages can help your physician understand if you have mild, moderate, severe or very severe PF. which is the amount of air that is exhaled starting from a maximal inhalation, how much air your lungs can hold and how easily it is for gases to move between your blood and lungs These percentages can help your physician understand if you have mild, moderate, severe or very severe PF.
• A six-minute walk test may be done to measure your exercise capacity. These tests can be compared over time to see if your lung disease has progressed and can help determine if you may need to use supplemental oxygen.
• A high-resolution CT scan (HRCT) shows how much scarring is in the lungs. These can be compared over time to see if scarring is worsening.