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. Healthcare providers 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) provide important information about how your lungs are working. PFTs measure your forced vital capacity (FVC), the amount of air you can exhale after taking a deep breath, as well as how much air your lungs can hold and how effectively oxygen moves from your lungs into your bloodstream. These results help your healthcare provider understand the severity of your pulmonary fibrosis and monitor changes in lung function over time.
• 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.