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What is pulmonary fibrosis?
1
Pulmonary fibrosis (PF) is a condition in which lung tissue becomes scarred,
thickened, and stiff. The scarring is called fibrosis and makes it hard for
a person to breathe.
Figure a
How does pulmonary fibrosis affect the lungs?
1
The air sacs and blood vessels in the lungs work to deliver oxygen to the body.
In people with PF, the tissue inside and between the air sacs becomes scarred and stiff.
The scarring and stiffness make it hard for oxygen to pass through the walls of the
air sacs into the bloodstream.
As a result, the brain, heart, and other vital organs may not get the oxygen
they need to work properly.
What causes pulmonary fibrosis?
PF can be caused by many things, some of the
known causes of PF include:
Job-related and environmental factors. Being
exposed to toxins and pollutants over the long term
can damage the lungs. These toxins and pollutants
may include silica dust, asbestos fibers, grain dust,
and some bird and animal droppings. 2,3,4
Radiation treatments. Radiation treatment for
lung or breast cancer may also damage the lungs
in some people. 3
Certain medicines. Some chemotherapy medicines
to treat cancer, some heart medicines, and some
antibiotics are known to cause lung damage.1
Certain medical conditions. Lung tissue can also
be damaged by conditions such as tuberculosis,
pneumonia, lupus, rheumatoid arthritis, sarcoidosis,
and scleroderma. 3,5,6,7,8
Genetics. Genes can play a role in the development
of PF. 2
When there is no known cause for PF, the disease
is called idiopathic pulmonary fibrosis, or IPF.1
For more information about the causes of PF,
visit www.pulmonaryfibrosis.org/causesofpf.
To request a copy of the Pulmonary Fibrosis Patient
Information Guide, contact the Pulmonary Fibrosis
Foundation at 888.733.6741 (from the US) or
+1 312.587.9272 (from outside the US) or
info @ pulmonaryfibrosis.org.
What is IPF?
IPF is a specific form of PF; it is also reported
to be the most common type of PF. 2
Genetics may play a role since IPF may occur
in several members of a family. 2
Some researchers have also suggested that
gastroesophageal reflux disease (GERD) may
have a role in the development of IPF. 2
Who gets idiopathic pulmonary fibrosis?
If you have been diagnosed with IPF, you are
not alone.
As many as 132,000 people in the United States
have idiopathic pulmonary fibrosis.9
IPF is most common in people 50 years
or older. 2
Figure d
Figure B
O2
it is important for people living
with a form of iPF to work closely
with their healthcare providers
to find ways to try and preserve
their overall health.
inside of
alveolus
alveolus wall
CO2
inside of capillary
Figure e
O2
inside of
alveolus
alveoli
alveolus wall
alveolus wall with iPF
capillary
CO2
Figure C
inside of capillary
How does idiopathic
pulmonary fibrosis make
you feel?
IPF symptoms develop over
time. The most common
symptoms of IPF are: 10
• Shortness of breath
• A dry, hacking cough
Other symptoms that may
occur over time include:
• Fast, shallow breathing
• Fatigue and weakness
• Aching muscles and joints
• Weight loss that cannot
be explained
• Clubbing (widening and
rounding) of the tips of
the fingers and toes
Figure a shows the location of
the lungs and airways in the body.
alveolus wall with iPF
Figure B shows a cross section
of a normal alveolar-capillary unit.
What can happen to your body
over time when you have idiopathic
pulmonary fibrosis?
IPF can progress slowly or quickly. It varies
from person to person and is hard to predict.2
In some people, IPF remains stable for years.
In others, symptoms get worse over time
and it becomes harder to breathe even when
you are not active.2
IPF can also lead to other medical problems.
As the disease gets worse, it can lead to respiratory (lung) failure, infections, high blood
pressure in the lungs (pulmonary hypertension),
and heart failure. 3
Figure C shows a cross section
of an alveolar-capillary unit with
thickening of the alveolar wall.
Figure d shows normal oxygen
exchange across the alveolus wall.
Figure e shows how oxygen
exchange across the alveolus wall
is impaired by fibrosis.1
How are PF and IPF treated?
The medications used to treat various forms of
PF will depend on its cause. Currently, in the US,
there are no FDA-approved treatments for people
with idiopathic pulmonary fibrosis (IPF). 11
Some treatments may help relieve the symptoms
of PF, and may help maintain your activity.
These treatments include:
Oxygen therapy. Oxygen may be prescribed if
the level of oxygen in your blood gets too low.
Using oxygen can help make breathing easier
and help you try to stay active.
It can also prevent or reduce problems from low
oxygen levels in your blood, reduce the blood
pressure in your heart, improve your sleep, and
help you feel better. 12
Pulmonary rehabilitation. Pulmonary rehabilita-
tion programs help people with PF. They focus
on physical exercise and breathing techniques.
They also provide nutritional counseling and
emotional support. 13,14
Lung transplantation. For some people with PF,
lung transplantation may be an option.15
Another option to consider is to enroll in a
clinical trial. Your healthcare provider will talk
with you about your options and which steps
might be right for you.
Who treats pulmonary fibrosis?
Finding a healthcare provider who has experience treating people with PF is important.
Pulmonologists (lung and breathing specialists)
familiar with PF can often be found at major
medical centers.
To find a medical center near you with expertise
in treating PF, call the Pulmonary Fibrosis
Foundation at 888.733.6741 (from the US)
or +1 312.587.9272 (from outside the US)
or visit www.pulmonaryfibrosis.org.
Can you benefit from participating in a clinical trial?
A clinical trial is a research study to learn more about investigational
treatments or new ways to use existing treatments. Some clinical trials
are performed to see if a new medicine is safe and effective for people
to use. Other clinical trials compare existing treatments to find out
which one is better.16
The US Food and Drug Administration (FDA), and other regulatory
agencies such as the European Medicines Agency (EMA), have regulations and guidelines to make sure people are provided with reliable
information to help them decide whether working with their doctor
to join a clinical trial is an appropriate option for them.17,18 Please be
sure to talk to your healthcare provider about all of your options.
Where can you find support?
In-person support groups
Participating in a support group may help individuals
with PF and family members better manage the
challenges of living with PF. 3
To find a support group near you, visit
www.pulmonaryfibrosis.org/supportgroups/local.
If there is no support group in your area and you
would like to start one, call the Pulmonary Fibrosis
Foundation at 888.733.6741 (from the US) or
+1 312.587.9272 (from outside the US) or visit
www.pulmonaryfibrosis.org/supportgroups/create.
Online support groups
Inspire. These online support groups are for people
with PF and their caregivers. Learn more about the
Pulmonary Fibrosis Foundation’s Inspire community
at www.inspire.com/partners/pulmonary-fibrosisfoundation.
RareConnect. This international online community
is designed to connect people with IPF who speak
different languages. Learn more about RareConnect
at www.rareconnect.org/en/community/idiopathicpulmonary-fibrosis.
How can you live better with pulmonary fibrosis?
Living with PF is not always easy. But there are some things that you can do to help manage
your condition and try to maintain your quality of life.19,20
Take an active role with your
Consider making some
care team
lifestyle changes
Your care team includes your healthcare
providers, your supporters, and YOU. Being
actively involved in your treatment is important
for individuals with PF.
Stay active and keep in shape.
Work closely with your healthcare providers
and others on your care team.
Call your healthcare provider if you do not feel
well or notice any unusual symptoms.
Take all of your medications exactly as directed
by your healthcare provider.
Follow your healthcare provider’s dietary and
exercise recommendations.
Get your vaccinations. Respiratory (lung)
infections like the flu can make your PF
symptoms worse.21 It is important for you to
talk with your doctor about getting a flu shot
every year and be up to date with your
pneumonia vaccine.
Eat well and maintain a healthy body
weight. A dietitian can help you create
a healthy eating plan that works for you.
Get plenty of rest.
Stop tobacco use and avoid secondhand
smoke.
Learn and practice relaxation techniques
to help manage stress.
Join a support group. Family members
and caregivers can join too.
Try to keep a positive attitude. If you feel
depressed or anxious, be sure to tell your
healthcare provider or another member
of your care team.
Keep all of your medical appointments.
Speak up for yourself. Talk with your health-
care providers about your questions or concerns.
Be prepared for your medical visits. Prepare
a list of questions to take to your appointments.
For a list of questions, visit www.pulmonaryfibrosis.org/yourappointment.
Take notes at your appointments. Or have
a friend or family member come with you to
take notes.
Get support and stay informed. Go to educa-
tional meetings held by your local medical
center, join a support group, and connect with
the Pulmonary Fibrosis Foundation.
Support for you from
the Pulmonary Fibrosis Foundation
The Pulmonary Fibrosis Foundation (PFF)
provides support for the entire PF community.
Some of the resources provided by the PFF include:
• Pulmonary Fibrosis Patient Information Guide
• Educational Programs
• Access to support groups
• Breathe Bulletin magazine
• Funding for research
To learn more about the Pulmonary Fibrosis
Foundation, call 888.733.6741 (from the US)
or +1 312.587.9272 (from outside the US) or visit
www.pulmonaryfibrosis.org.
references
1NHLBI, NIH. What Is Idiopathic Pulmonary Fibrosis?
Accessed at: http://www.nhlbi.nih.gov/health/health-topics/
topics/ipf/. Accessed July 2013
2Raghu G, Collard H, et al. An Official ATS/ERS/JRS/ALAT
Statement: Idiopathic Pulmonary Fibrosis: Evidence-based
Guidelines for Diagnosis and Management. Am J RespirCrit
Care Med. 2011; 183: 788–824, 2011
3 Schwarz, M.I and King, T.E. Interstitial Lung Disease,
5th Edition. (Shelton, CT: People’s Medical Publishing HouseUSA). 2011.
4 Chan AL et al. Clinic Rev Allerg Immunol (2012) 43:69–83.
DOI 10.1007/s12016-011-8282
5Dheda K et al. Lung Remodeling in Pulmonary Tuberculosis.
J Infect Dis. (2005) 192 (7): 1201–1210. doi: 10.1086/444545
6 Cheema GS, Quismorio FP Jr. Interstitial lung disease in
systemic lupus erythematosus. Curr Opin Pulm Med. 2000
Sep;6(5):424–9.
12 Wells, A. U. Interstitial lung disease guideline: the British
Thoracic Society in collaboration with the Thoracic Society of
Australia and New Zealand and the Irish Thoracic Society.
Thorax 2008;63(Suppl V):v1–v58. doi:10.1136/thx.2008.
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13 Jastrzebski,D. et al. Dyspnea And Quality Of Life In Patients
With Pulmonary Fibrosis After Six Weeks Of Respiratory
Rehabilitation. Journal Of Physiology And Pharmacology
2006, 57, Supp 4, 139 148
14 Spruit, et al. An Official American Thoracic Society/European
Respiratory Society Statement: Key Concepts and Advances
in Pulmonary Rehabilitation. Am J Respir Crit Care Med Vol
188, Iss. 8, pp e13–e64, October 15, 2013
15 International Guidelines for the Selection of Lung Transplant
Candidates: 2006 Update — A Consensus Report from the
ISHLT Pulmonary Scientific Council (Consensus Document).
J Heart Lung Transplant 2006;25(7):745–755
7 Tanoue LT. Pulmonary manifestations of rheumatoid arthritis.
Clin Chest Med. UID 9917959.
16 Learn About Clinical Studies. Clinical Trials.gov. Available at:
http://clinicaltrials.gov/ct2/about-studies/learn. Accessed
November 2013.
8Le Pavec J. et al. Scleroderma lung disease. Clin Rev Allergy
Immunol. 2011 Apr;40(2):104–16. doi: 10.1007/s12016009-8194-2
17 About FDA — What Does FDA do? FDA.gov. Available at:
http://www.fda.gov/AboutFDA/Transparency/Basics/ucm
194877.htm. Accessed November 2013
9 Raghu, G. Incidence and Prevalence of Idiopathic Pulmonary
Fibrosis. American Journal of Respiratory and Critical Care
Medicine, Vol. 174, No. 7 (2006), pp. 810–816. doi: 10.1164/
rccm.200602-163OC. Available at: http://www.atsjournals.org/
doi/full/10.1164/rccm.200602-163OC. Accessed August 2013
18 What we do. European Medicines Agency. Available at: http://
www.ema.europa.eu/ema/index.jsp?curl=pages/about_us/
general/general_content_000091.jsp&mid=WC0b01ac
0580028a42. Accessed November 2013
10 Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis.
Orphanet J Rare Dis. 2008 Mar 26;3:8. doi: 10.1186/17501172-3-8. Accessed at: http://www.ojrd.com/content/3/1/8.
Accessed July 2013
11 Zolak, JS, de Andrade, JA. Idiopathic Pulmonary Fibrosis.
Immunology and Allergy Clinics of North America, Volume 32,
Issue 4, November 2012, Pages 473–485
19 Florian, J. et al. Impact of pulmonary rehabilitation on quality
of life and functional capacity in patients on waiting lists
for lung transplantation. J Bras Pneumol. 2013 Jun;39(3):
349–56. doi: 10.1590/S1806-37132013000300012.
20 Holland A, Hill C. Physical training for interstitial lung disease.
Cochrane Database Syst Rev. 2008 Oct 8;(4):CD006322.
doi: 10.1002/14651858.CD006322.pub2.
21 Rothberg MB et al. Complications of Viral Influenza.
The American Journal of Medicine (2008) 121, 258–264
about the Pulmonary Fibrosis Foundation
The mission of the Pulmonary Fibrosis Foundation (PFF) is to help find a cure for
idiopathic pulmonary fibrosis (IPF), advocate for the pulmonary fibrosis (PF) community,
promote disease awareness, and provide a compassionate environment for patients
and their families.
Pulmonary Fibrosis Foundation
230 East Ohio Street, Suite 304
Chicago, Illinois 60611-3201
888.733.6741 (from the US) or
+1 312.587.9272 (from outside the US)
www.pulmonaryfibrosis.org
© 2013 Pulmonary Fibrosis Foundation. All rights reserved.
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