Pulmonary Fibrosis Stages

If you’re living with a chronic lung disease like pulmonary fibrosis (PF)interstitial lung disease (ILD) or chronic obstructive pulmonary disease (COPD), you’ve likely heard your doctor talk about stages.

For people with COPD, the GOLD System and the BODE Index are often used to place COPD into stages, ranking it by disease severity.

The GOLD System uses the forced expiratory volume (FEV1) score from a pulmonary function test to determine the stage of COPD. The BODE Index takes into account multiple test results and procedures to determine a COPD stage.

However, for people living with pulmonary fibrosis, the pulmonary fibrosis stages are not as well defined. To help you better understand your condition, here’s what you need to know about pulmonary fibrosis stages and what’s being done to help you breathe easier.

What is pulmonary fibrosis?

If you’ve been diagnosed with pulmonary fibrosis or idiopathic pulmonary fibrosis, you’ve probably heard your doctor talk about lung scarring.

In pulmonary fibrosis, the lungs become scarred, which causes the intricate pulmonary passageways to thicken and harden.

When the lungs thicken and harden, the free passage of oxygen through the walls of the lungs’ tiny air sacs (alveoli) into the bloodstream becomes obstructed.

Because of pulmonary fibrosis disease progression, the resulting lack of oxygen in the bloodstream leaves people with pulmonary fibrosis feeling short of breath even after prolonged rest.

Pulmonary fibrosis affects everyone differently and at different rates of disease progression. For some people, pulmonary fibrosis develops rapidly, and for others it progresses slowly over time.

How are the pulmonary fibrosis stages determined?

In general, having a staging system for a particular disease helps doctors and patients work together to develop a treatment plan.

Traditionally, pulmonary fibrosis has been in stages with terms such as mild, early, severe and advanced. These simple pulmonary fibrosis stages are based on pulmonary function test results.

While this approach allows doctors to monitor pulmonary fibrosis disease progression and helps them advise patients, the current pulmonary fibrosis stages approach doesn’t take into account certain variables.

Some of the baseline features of the disease that could be useful in developing a better system for pulmonary fibrosis stages include level of dyspnea (trouble breathing), forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide, desaturation during the 6-minute walk test, extent of honeycombing appearance of the lungs of CT scans or the presence of pulmonary hypertension or emphysema.

New developments in pulmonary fibrosis staging

Doctors and researchers have been developing a new staging system for pulmonary fibrosis. The new pulmonary fibrosis stages system currently in development is called the GAP Index.

More sophisticated than the traditional pulmonary fibrosis staging system, the GAP Index is comprised of four predictors: age, recent respiratory hospitalization, baseline FVC and 24-week change in FVC. 

The sum of the individual scores for each factor is then used to obtain a score.

More recently, doctors and researchers have continued to develop the GAP Index, using a multidimensional risk prediction model and staging system. 

This Gap Index model also consists of 4 baseline variables: gender, age, and two lung physiology variables (FVC and diffusing capacity of the lung for carbon monoxide). 

They were able to develop a GAP Index calculator to help doctors and patients better understand pulmonary fibrosis stages, where a patient might fall within those stages and the individual risk for each patient.

There are GAP Index calculators available online. Remember to discuss your GAP Index results with your doctor as well as any changes to your symptoms.

What pulmonary fibrosis treatment options are available?

Now that you know more about the pulmonary fibrosis stages and the new developments in better staging systems, you and your doctor can work together to develop the best treatment plan for you.

While there’s not a cure for pulmonary fibrosis, there are treatment options available.

Many doctors prescribe medications, inhalers and oxygen therapy to help people with pulmonary fibrosis manage their symptoms.