Idiopathic Pulmonary Fibrosis in Canada: Facts and Care

Idiopathic pulmonary fibrosis, or IPF, is scarring of lung tissue. It stiffens the part of the lung where oxygen crosses into the blood, so breathing takes more effort. Day to day that means breathlessness on stairs or hills, and a dry cough that will not clear.

By the numbers in Canada

More than 14,000people in Canada live with idiopathic pulmonary fibrosis, according to the Canadian Pulmonary Fibrosis Foundation's report on the burden of IPF in Canada, which drew on national administrative data recording 14,259 cases, or 41.8 per 100,000 people.

Source: Canadian Pulmonary Fibrosis Foundation, The Burden of Idiopathic Pulmonary Fibrosis in Canada, 2019

Diagnosis is often slow. The same report states that people may be symptomatic for more than five years before diagnosis, with at least a one-year delay between the onset of breathing problems and a diagnosis, and that more than one third saw three or more physicians first. Before the correct diagnosis, IPF had been labelled as bronchitis in 48 percent of cases, asthma in 18 percent, COPD in 14 percent and emphysema in 8 percent. Source: Canadian Pulmonary Fibrosis Foundation, 2019
Where you live changes what care you can reach. The report counted 16 interstitial lung disease centres across Canada, and respirologists ranging from 3.0 per 100,000 people in Quebec and 2.0 in Ontario to none in the territories. It documents differences between provinces in access to home oxygen and pulmonary rehabilitation, and notes that provincial coverage of antifibrotic medicines began at different times between 2014 and 2018. Source: Canadian Pulmonary Fibrosis Foundation, 2019
IPF is a leading reason for lung transplantation in Canada, accounting for 46.1 percent of single lung transplants and 26.4 percent of bilateral lung transplants. Average direct health costs were reported at slightly under $13,000 per year after diagnosis, with one-time diagnostic costs of slightly more than $15,000. Source: Canadian Pulmonary Fibrosis Foundation, 2019

Worldwide390,267 new cases of interstitial lung disease and pulmonary sarcoidosis, the wider group that includes IPF, were recorded worldwide in 2021 in the Global Burden of Disease study, along with 188,222 deaths. This is an international figure, not a Canadian one.Source: Global Burden of Disease Study 2021, Frontiers in Public Health, 2025

What IPF is, and what it is not

Idiopathic means the cause has not been identified. Lung scarring can follow asbestos or bird dust, an autoimmune condition or certain medicines; when none of those are found and a CT scan shows a particular pattern, it is IPF. It is not an infection, COPD or asthma, though the Canadian Pulmonary Fibrosis Foundation reports it is often labelled as one of those first.

It is a serious condition that gets worse over time in most people, at a pace that varies widely. Some stay stable for long stretches, others decline faster, and no one can say at diagnosis which pattern yours will follow. Scarring already present does not reverse, so care aims at slowing further change and supporting your breathing.

Signs and symptoms

Breathlessness, at first only on stairs or hills
A dry cough lasting more than eight weeks
Tiredness and low energy
Low appetite and unexplained weight loss
Changes in the shape of the fingertips, called clubbing

How fast symptoms build differs between people.

A woman standing by an open balcony door with houseplants, looking outside on a bright day. — Breathlessness on stairs or hills is often the first thing people notice. Illustrative photograph.

What raises the risk

Some risk factors cannot be changed:

Age over 50
Being male, which the Canadian Lung Association reports in about 75 percent of people with IPF
A family history of interstitial lung disease, present in up to 20 percent of cases

Others can often be worked on, usually with support from a health care team:

Cigarette smoking, reported in about 75 percent of people with IPF
Acid reflux, also reported in about 75 percent, which a care team can treat
Exposure to dusts, fumes and smoke, including wildfire smoke

How IPF is diagnosed in Canada

IPF is diagnosed by a respirologist, usually with an interstitial lung disease clinic, and by a team weighing several results together rather than any single test. The Canadian Lung Association lists the tests: pulmonary function tests, a six-minute walk test, a chest x-ray, blood tests for other causes of scarring, and a computed tomography scan of the chest, the key imaging test. Some people also have a bronchoscopy or a surgical lung biopsy where the picture stays unclear.

Treatment and day-to-day management

Care aims to slow the disease and support your breathing. A respirology team may discuss:

Antifibrotic medicines, nintedanib and pirfenidone, approved in Canada and aimed at slowing the formation of scar tissue. Both are covered by provincial and territorial drug programs and by First Nations Non-Insured Health Benefits
Pulmonary rehabilitation, a supervised exercise and education programme with respiratory therapists
Supplemental oxygen at home if oxygen levels fall
Treating reflux and managing cough
Assessment for lung transplantation at a transplant centre
Immunosuppressant medicines such as prednisone and mycophenolate, used mainly for other forms of fibrosis and for flares

Clinical trials are listed alongside these. What fits you is a decision for you and your own respirologist.

When to talk to a doctor

Breathlessness worsening over hours or days, a new fever, or a change in phlegm needs urgent assessment: a flare of IPF is treated as an emergency. Call 911 if you are struggling to breathe, your lips or face look blue or grey, or you feel confused.
Chest pain or coughing up blood needs prompt assessment.
New ankle swelling, or home oxygen readings lower than usual, should be reported promptly.
Breathlessness building over months, or a dry cough lasting more than eight weeks, is worth an appointment now.

Why clinical research matters for IPF

The cause is unknown, nothing available reverses scarring already formed, and there is no reliable way to predict how fast the disease will move. Those gaps drive the research. Studies are testing new antifibrotic and anti-inflammatory agents, inhaled treatments, combinations with the two medicines approved in Canada, and treatments for the cough and breathlessness that affect daily life most. Others focus on finding IPF earlier, since the Canadian Pulmonary Fibrosis Foundation documented delays of a year or more before diagnosis.

Taking part is voluntary, informed consent comes first, you can stop at any time without affecting your regular care, and no one can say in advance whether a study will help you.

Learn more from Canadian sources

Where this information comes from (4 sources)

This page is written in plain language for people considering clinical research. It is general health information, not medical advice, and it does not replace a conversation with your own doctor or nurse practitioner.

Common questions

What does idiopathic mean, and does it mean no one knows anything?

Idiopathic means the cause has not been identified. Lung scarring can follow a known exposure such as asbestos or bird dust, an autoimmune condition, or certain medicines. When the scarring shows a particular pattern on a CT scan and none of those causes are found, it is called idiopathic pulmonary fibrosis. A great deal is known about how IPF behaves, how it is diagnosed and what care exists. What is not known is what starts it.

Why was I told I had bronchitis or COPD first?

Because the early symptoms overlap. Breathlessness on exertion and a dry cough are far more often caused by common conditions than by IPF, which is uncommon. The Canadian Pulmonary Fibrosis Foundation found that before the correct diagnosis, IPF had been labelled as bronchitis in 48 percent of cases, asthma in 18 percent and COPD in 14 percent, that more than one third of people saw three or more physicians first, and that there was at least a one-year delay between the first breathing problems and diagnosis. What usually settles it is a CT scan of the chest read alongside breathing tests.

Why would someone with IPF join a clinical trial?

Because the two antifibrotic medicines approved in Canada are aimed at slowing the disease rather than reversing it, and a great deal is still being tested: new agents, inhaled treatments, combinations, and treatments for cough and breathlessness. The Canadian Pulmonary Fibrosis Foundation lists clinical trials alongside the other parts of care. Taking part is voluntary, you give informed consent before anything begins, you can withdraw at any time without affecting the care you get from your respirologist, and no one can say in advance whether a study will help you personally.

What to do next

Talk to your doctor. This guide is information, not medical advice.
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Idiopathic Pulmonary Fibrosis (IPF)