People living with pulmonary fibrosis
often experience confusion and frustration
as they try to understand their diagnosis.
Pulmonary fibrosis is a complicated diagnosis
that encompasses many diseases.
The big picture of lung disease is vast
and includes diagnoses such as COPD, asthma, and cystic fibrosis.
In addition, we have a family of diseases called ILD or Interstitial Lung Diseases.
Now, there are over one hundred different kinds
of Interstitial Lung Diseases.
We group these disease together
because they share two common features.
One is inflammation
and the other is scar tissue.
These both occur in the walls
of the tiny air sacs of the lungs.
Your lung is made up of
millions of tiny air sacs
too small to see without a microscope.
The wall of each air sac is very thin -
about ten times thinner than a strand of hair.
These walls need to be thin
to allow oxygen inside the air sac
to move easily through the wall into the bloodstream.
It’s easy to take a breath as thin walls
mean that the lung is flexible
and, therefore, easy to expand when you breathe.
When scar tissue or inflammation builds up
in the walls of the air sacs, the wall gets thicker,
making it more difficult for oxygen to get into the bloodstream.
Thickened walls are also stiff making it harder to breath
which causes breathlessness.
Scar tissue and inflammation can also trigger cough.
So, what is the difference between interstitial lung disease
and pulmonary fibrosis?
Interstitial lung diseases include all of the diseases in which there is inflammation
or scar tissue in the walls of the air sacs.
Many forms of interstitial lung disease can
also be called pulmonary fibrosis.
Pulmonary fibrosis however is not a specific disease,
it is a general term that refers
to any kind of ILD in which scar tissue is present
in the walls of the air sacs.
Your doctor will work with you to figure out which kind of ILD you have.
Many people know that asbestos and working in a coal mine can damage the lung.
These are examples of pulmonary fibrosis
due to exposures in the workplace.
One of the most common forms of pulmonary fibrosis
is called chronic hypersensitivity pneumonitis.
This is a long way of saying that
the lung is too sensitive to airborne particles that
cause inflammation and scarring in the lung.
Airborne mold spores and bacteria in the home,
workplace, or other indoor environments,
and exposure to birds
are common causes of chronic hypersensitivity pneumonitis.
Other causes of pulmonary fibrosis include
certain medications including amiodarone for cardiac arrhythmias,
nitrofurantoin for urinary tract infections,
and chemotherapy for different forms of cancer.
Radiation treatment for cancers in the chest can also lead to pulmonary fibrosis.
People who have autoimmune conditions like rheumatoid arthritis,
or scleroderma are at risk for pulmonary fibrosis.
Many people have these exposures
but don’t develop PF.
Often, however, doctors won’t be able to
identify a specific cause of your disease.
If this is the case for you,
you might have been told your disease was idiopathic.
The word “idiopathic” means of unknown cause.
You may have heard of the disease “idiopathic pulmonary fibrosis.”
This is one kind of pulmonary fibrosis
of unknown cause but you may not be aware
that there are many other types of pulmonary fibrosis of unknown cause
including idiopathic non-specific interstitial pneumonia, sometimes called NSIP,
pulmonary sarcoidosis, and cryptogenic organizing pneumonia.
The Pulmonary Fibrosis Foundation offers
essential programs and resources for individuals living
with pulmonary fibrosis.
Our mission is to mobilize people and resources
to provide access to high quality care, and
to lead research for a cure so that people with PF
will live longer, healthier lives.
For more information on this,
please visit pulmonaryfibrosis.org.