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.

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