The Two Main Types of Pulmonary Fibrosis


Pulmonary fibrosis is a chronic, progressive lung disease where the tissue in the lungs becomes scarred and thickens over time. This thickening (or fibrosis) leads to stiffness in the lungs which makes it difficult for oxygen to pass through the air sacs (alveoli) and into the blood stream.

There are two main types of pulmonary fibrosis:

Idiopathic Pulmonary Fibrosis
The term “idiopathic” means the cause of the disease cannot be confirmed. Because the underlying source of the disease is unknown, the disease can be difficult to treat. Currently, there are two medications approved bu the FDA for treating idiopathic pulmonary fibrosis (IPF) — Esbriet and Ofev — which can help slow down the progression of the disease and avoid exacerbations.

Respiratory failure is the primary concern for IPF patients, but the disease can also lead to heart failure, pulmonary hypertension, pneumonia and lung cancer.

MORE: What you need to know about Esbriet.

Familial Pulmonary Fibrosis
Familial pulmonary fibrosis is very rare. While it’s just as serious as idiopathic pulmonary fibrosis, familial PF is usually diagnosed much earlier which gives the patient a better prognosis.

The different stages of both types of pulmonary fibrosis can be broken down into mild, moderate, severe and very severe.

MORE: The role genetics play in pulmonary fibrosis.

Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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  1. Laura says:

    I wish ( broken record here) that all these articles would state that these meds are NOT good for those complicated by COPD as well.

    • Doyla lane says:

      Can you explain I was on Esbriet for IPF and also have copd.I was taken off of it few months ago due to bloodwork issues but should be able to start it back and have never been informed that it wasn’t good for ones who also had copd

  2. ed mier says:

    It would have been nice to spell out the definition of mild, moderate, severe and very severe IPF. We would like to know where we stand in relation to our colleagues with the disease. Just a “nice to know” thing.

  3. Peter Wriedt says:

    I was recently diagnosed as having suffered from carbon monoxide poisoning and severe smoke inhalation. I am having problems with breathing and just walking up stairs is extremely difficult for breathing. I can’t get my family doctor to refer me to pulmonary doctor for stress test and checking internally my lungs to find out why my breathing keeps getting worse. Appreciate any help to get help with my health condition.

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