What Are the Stages of Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis (IPF) is a rare, progressive lung disease. While there are not formal categories of IPF, doctors and patients sometimes think of IPF in four different stages depending on symptoms and treatment needs. 

IPF can be a scary diagnosis, but it's not a death sentence. IPF progresses differently for everyone. Some live with the disease for many years. As the disease progresses, your treatment needs will change, but it's important to continue to work closely with your doctor, stay as active as possible, and take care of your mental health, too.

Stage 1: Recently diagnosed

Early symptoms of IPF can include fatigue, shortness of breath with activity, and a dry cough. Some people with stage 1 IPF may not show symptoms at all, or only experience them with extreme exertion, such as climbing several flights of stairs. 

When you're first diagnosed with IPF, doctors may also conduct a series of tests to understand disease progression and decide on treatment options. They may ask questions about symptoms, particularly shortness of breath and cough. Pulmonary Function Tests (PFTs) or Lung Function Tests show doctors how much air the lungs can hold, and how forcefully someone can push air out. They may also conduct a six-minute walk test to measure how much you can exercise and your shortness of breath during activity. 

Treatments at this stage include the two FDA-approved treatments for IPF, Esbriet and OFEV, and lifestyle changes such as quitting smoking and continuing to exercise.

Stage 2: Some oxygen needed with activity, but not at rest

As IPF progresses, shortness of breath with activity becomes more common. Coughing is typically more common at this stage as well. 

Doctors prescribe patients oxygen to use with activity at this stage, based on oxygen saturations with rest and activity. Patients can monitor their oxygen saturation with a pulse oximeter to make sure the flow is correct, maintaining a saturation equal to or greater than 89%.

While it may feel more difficult, it's important to continue exercising at this stage. Oxygen with activity should help you feel better. 

Stage 3: Needing oxygen 24 hours a day 

At this stage, patients feel shortness of breath with activity. Their oxygen levels are low enough to require oxygen at rest, but they will not feel short of breath while at rest. Cough and fatigue can continue to be bothersome. 

Exercise can continue to help those with IPF improve energy levels and symptoms, even with 24-hour oxygen. 

Stage 4: Advanced oxygen needs (high-flow oxygen when a portable, lightweight oxygen machine no longer meets patient needs)

When a portable, lightweight oxygen delivery system no longer meets a patient's needs, doctors will recommend a high-flow oxygen in a non-portable delivery system. 

High-dose oxygen can't be delivered by portable oxygen containers, so those at this stage may feel isolated socially. It's important for family and friends to continue to offer support, and for those living with IPF to talk to their doctors if they're feeling depressed.

Idiopathic Pulmonary Fibrosis clinical trials

Currently, there are no IPF treatments that stop disease progression. Clinical trials for IPF research potential new treatments, and need volunteers. Interested in finding a trial near you? Answer a few questions to start.