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Stages of Idiopathic Pulmonary Fibrosis:


There is no formal staging system for Idiopathic Pulmonary Fibrosis. However, various organizations will describe an informal systems.  A couple are listed below.

National Jewish Health  believes thinking of IPF in four stages can help people with IPF prioritize their needs and keep them from feeling overwhelmed. Below the National Jewish Health organization describes their four stages of IPF:

Stage 1: Recently diagnosed

Stage 2: Needing oxygen with activity, but not at rest

Stage 3: Needing oxygen 24 hours a day, with activity, at rest and during sleep

Stage 4: Advanced oxygen needs (needing high-flow oxygen or when a lightweight, portable delivery system is unable to meet a patient’s needs).

The American Lung Association states "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 doctor 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 helps your doctor better understand your particular disease.

Pulmonary Function Tests (PFTs) or Lung Function Tests give your doctor important information about the amount of air the lungs can hold and how forcefully you can empty air from the lungs. A PFT reveals your forced vital capacity (FVC) which is the amount of air that is exhaled starting from a maximal inhalation. This percentage can help your physician understand if you have mild, moderate, severe or very severe PF. The most basic test is spirometry.

A six-minute walk test may be done to measure your exercise capacity.

A high-resolution CT scan (HRCT) shows how much scarring is in the lungs."

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