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  • Lung Condition

Pulmonary Fibrosis

Pulmonary fibrosis (also known as interstitial lung disease or ILD) is a chronic (long-term) disease where the lungs (pulmonary) become scarred (fibrosis). The scarring causes the part of the lung where oxygen is moved to the blood (interstitial) to become thick and stiff. If the reason or cause of the disease is unknown, it is called idiopathic.

Pulmonary fibrosis is a restrictive disease because it is hard to fully expand the lungs when taking a breath in (i.e., the lungs are restricted when taking a breath in).

14–15K

Canadians affected by IPF

IPF

Most common interstitial lung disease

50+

More common in males over age 50

Chronic

Long-term, progressive condition

  • Symptoms
  • Causes
  • Risk factors for IPF
  • Diagnosis
  • Treatment

Symptoms

Recognising pulmonary fibrosis

We often don’t think about our breathing until something takes our breath away. When the lungs become scarred, for example in pulmonary fibrosis, it is hard to get oxygen to the organs and the rest of the body. The lack of oxygen can cause different symptoms. Knowing what to look for is important so you and your healthcare provider can come up with a plan to diagnose the problem.

People with pulmonary fibrosis may have some or all the following symptoms:

Shortness of breath

Especially during or after physical activity

A dry cough

Inspiratory crackles

A 'velcro-like' crackling sound in the lungs that can be heard when listening with a stethoscope

Toe/finger clubbing

About one half of people with idiopathic pulmonary fibrosis or IPF have rounding and widening of the fingers and toes

Unintended weight loss and feeling unwell

Fatigue and weakness

  • Speak with your healthcare provider
 Speak with your healthcare provider if you have these symptoms.

Causes

What causes pulmonary fibrosis?

There are several known causes of pulmonary fibrosis, as well as unknown causes. Known causes of lung scarring (fibrosis) include:

  • Medical tests and treatments Certain medications such as for inflammation (anti-inflammatory), infections (antibiotic), and cancer (chemotherapy) can cause scarring in the lungs. Medical tests like radiation therapy are also linked to pulmonary fibrosis.
    Medical tests and treatments
  • Occupational or environmental exposures Inhaling toxic compounds such as coal (coal workers' pneumoconiosis), silica (silicosis), and asbestos (asbestosis) can cause scarring in the lungs that may lead to pulmonary fibrosis. Also, certain inhaled drugs or chemicals such as cocaine, zinc chloride, and ammonia are known to cause scarring in the lungs.
    Occupational or environmental exposures
  • Autoimmune disease There is some proof that pulmonary fibrosis can be caused by autoimmune diseases like rheumatoid arthritis, scleroderma, and systemic lupus erythematosus (SLE). In autoimmune disease the body's natural defense system mistakenly attacks normal cells and causes the disease.
    Autoimmune disease
  • Idiopathic pulmonary fibrosis (IPF)
When no cause for pulmonary fibrosis can be identified the disease is termed idiopathic pulmonary fibrosis or IPF. Idiopathic simply means ‘of unknown cause’.

Risk factors for IPF

Who is at risk for IPF?

Although IPF is a disease of unknown cause, there are several potential risk factors for the development of IPF that are known. They include:

  • Smoking Smoking is one of the most recognized risk factors.
    Smoking
  • Environmental exposures Certain jobs and occupations such as farming, raising birds, hair dressing, and stone cutting/polishing may be connected. Contact with livestock and different kinds of dust (metal, wood, vegetable and animal) are also risk factors.
    Environmental exposures
  • Gastroesophageal Reflux Disease (GERD) GERD is a chronic digestive disease. GERD occurs when stomach acid or content flows back into your food pipe (esophagus). This backwash (reflux) irritates the lining of your esophagus and is what causes GERD. GERD may also be related to IPF, however more research is still needed in this area.
    Gastroesophageal Reflux Disease (GERD)
  • Genetics Genetics can play a role in IPF. A very small percentage of people with IPF have reported two or more family members with the disease.
    Genetics
  • Gender IPF is more common in men than women.
    Gender
  • Age IPF is more common in people 50 years and older. The risk of getting IPF for both women and men increases with age.
    Age

Diagnosis

How pulmonary fibrosis is diagnosed

There is no single test to diagnose pulmonary fibrosis, so it may take some time to determine if you have the disease. You will likely need to undergo many different tests and often healthcare providers will first rule out other possible diseases. They often start with tests that are the least invasive and only order more if needed. These tests will help toward a better understanding of your condition.

Below are some of the steps and tests that will help your healthcare provider make the right diagnosis for you.

First step

Medical History

Your healthcare provider will ask about you and your family's health. They will want to know any symptoms you may be having (e.g. coughing or shortness of breath during physical activity) and if you have any other medical conditions.

Exam

Physical Examination

Your healthcare provider will check for any visible signs and symptoms that could indicate pulmonary fibrosis.

Blood oxygen

Oxygen Testing

This test measures the oxygen in your blood (oxygen saturation). Your healthcare provider will clip a 'probe' to your finger to measure and monitor how much oxygen is in your blood. This alone does not diagnose IPF but can be one of the tests that assists with a diagnosis.

Blood Test

Arterial Blood Gases (ABGs)

ABGs measure the amount of oxygen that is carried in your blood. A blood sample is usually taken from an artery in your wrist.

Breathing

Pulmonary Function Tests (PFTs)

PFTs or 'breathing tests' are a series of tests used to find out how well you move air in and out of your lungs and how well oxygen enters your bloodstream from your lungs.

Exercise

Six Minute Walk Test (6MWT)

This test measures how far you can walk in six minutes, how difficult it was for you to walk in the six minutes, and if your oxygen levels dropped while walking.

Blood

Blood Test

Although a blood test cannot confirm pulmonary fibrosis, it can be used to help rule out other lung-related conditions.

Imaging

Chest Radiography (x-ray)

A chest x-ray uses electromagnetic waves to create pictures of the structures in and around your chest. It will often provide useful information such as the size of your lungs and can also help rule out other lung diseases.

Airway

Bronchoscopy

A bronchoscopy allows the doctor to look through your nose or mouth into the trachea (windpipe) and down to the bronchi (large airways) in your lungs.

Imaging

High Resolution Computed Tomography (HRCT)

Also known as a CAT or CT scan, these scanners are able to take more detailed x-rays of the chest.

Tissue

Lung Biopsy

A lung biopsy involves small incisions into the side of the chest to remove a small part of the lung tissue and examine it under a microscope.

Treatment

Treatment options

Currently there are two approved drugs in Canada for the treatment of pulmonary fibrosis (IPF), which is the most common form of pulmonary fibrosis. These medications are called anti-fibrotic agents, meaning they are used to slow down the rate of fibrosis or scarring in the lungs.

Nintedanib

Ofev®

Pirfenidone

Esbriet®, Sandoz®

  • For information about medication coverage in Alberta
Interactive Drug Benefit List  ·  Special Authorization Request Form

Other treatments

  • Supplemental Oxygen Therapy Can be prescribed by a healthcare provider. It is not a cure for pulmonary fibrosis, and it will not take away your shortness of breath. However, it will treat low blood oxygen levels that can occur when pulmonary fibrosis worsens. Oxygen in the blood is needed for all organs, including your brain to repair and function.
    Supplemental Oxygen Therapy
  • Steroids There are certain types of pulmonary fibrosis (excluding IPF) that can be treated using steroids. Steroids are a type of medication that reduce inflammation (swelling).
    Steroids
  • Medications for acid reflux Gastroesophageal reflux disease (GERD) is a digestive condition that commonly occurs in people with IPF. There are different types of anti-acid medications that might be prescribed to help block the formation (or lessen) the amount of acid in the stomach.
    Medications for acid reflux
  • Medications for cough A dry cough is a common symptom of pulmonary fibrosis. Because what works for one person might not work for another, treating a dry cough can be trial and error. Medications for cough might include over the counter medication or prescription cough medication.
    Medications for cough

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