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Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, the scarring gets worst and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen.

IPF is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis, and these are treated differently. It is important to work with your doctor to determine if you have IPF or another form of interstitial lung disease.

Causes

The cause of idiopathic pulmonary fibrosis is not completely understood. No one knows what causes idiopathic pulmonary fibrosis or why some people get it.

Symptoms

The two main symptoms of IPF are Breathlessness and Chronic Cough.

Diagnosis

Idiopathic pulmonary fibrosis (IPF) can be difficult to diagnose since many lung diseases can cause breathlessness and cough, and some of these other diseases can have similar symptoms and X-ray results.

Treatment

There is no cure for IPF and there are currently no procedures or medications that can remove the scarring from the lungs. Treatments are used to slow progression of the lung scarring, and may not necessarily lessen the symptoms of cough and breathlessness.

Managing Your IPF

It is important to work with your family doctor or respirologist (lung specialist) to find the best treatment for you. Not all medications will work for everyone.

Research and Trials

Researchers are conducting clinical trials in Canada and around the world.

Additional Resources

For information on how to set up an IPF education and support group, visit the Canadian Pulmonary Fibrosis Foundation website where you can download a toolkit.