Doctors stage a patient’s pulmonary fibrosis progression in order to better understand, monitor, and communicate the advance of the disease; make the best treatment decisions possible; and offer an accurate prognosis.

Traditional staging for pulmonary fibrosis is determined by how a patient scores on a forced vital capacity (FVC) test.  The test is a simple one that takes only about three minutes to complete. Patients inhale and then exhale for a sustained period of time into a spirometry device that measures maximal expiration and maximal inspiration (sustained breathing out followed by inhalation).

IPF severity is determined based on spirometry scores. A predicted FVC value of greater than 75% is considered mild, 50% to 75% is considered moderate, 25% to 49% is severe, and less than 25% is deemed very severe.

Limitations to pulmonary fibrosis stages scale

While the traditional pulmonary fibrosis staging system has been used by doctors for many years, some researchers believe that there are diagnostic limitations to its use. In spite of FVC ratings, traditional staging can sometimes appear arbitrary and not based on epidemiological or biological data. And while the use of these stages can help to approximate the progression of pulmonary fibrosis, it remains to be seen if the stages are truly relevant for disease management because the progression of pulmonary fibrosis is not linear and each patient has a unique, individual experience.

In an attempt to improve pulmonary fibrosis staging, researchers are developing new staging methods that will likely include factors such as age, recent respiratory hospitalization, baseline FVC, 24-week change in FVC, and possible biomarkers. Another staging technique, known as the GAP model, takes into account gender (G), age (A), and two lung physiology (P) variables (forced vital capacity and diffusing capacity of the lung for carbon monoxide).

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  1. http://pulmonaryfibrosismd.com/stages-of-idiopathic-pulmonary-fibrosis/
  2. http://err.ersjournals.com/content/23/132/220