Pulmonary fibrosis
DR DANIYA NOREEN Pharm–D Mphill RPH
Lecturer JSMU
overview
Pulmonary fibrosis is a serious, lifelong lung disease. It
causes lung scarring (tissues scar and thicken over time),
making it harder to breathe.
group of serious lung diseases that affect the
respiratory system. Pulmonary fibrosis scars and thickens
lung tissue. It impacts the connecting tissue in the lung and
the alveoli (air sacs inside the lungs).
The lung damage gradually gets worse over time. Hard, stiff
lung tissues don’t expand as well as they should, making it
harder to breathe. Pulmonary fibrosis may cause shortness
of breath when you do routine tasks
In pulmonary fibrosis, the thin walls of these air sacs start to
scar and thicken. When that happens, it’s harder for the air
sacs to do their job and get oxygen to the rest of the body.
• Is pulmonary fibrosis a terminal illness?
• Are pulmonary fibrosis and COPD the same?
• Providers classify pulmonary fibrosis as an interstitial lung disease.
The interstitial tissues are cells that make up the space between blood
vessels and other structures inside the lungs. Pulmonary fibrosis
damages these cells. It is a rare disease
• COPD is a more common type of lung disease. Diseases like
emphysema and chronic bronchitis are types of COPD. In COPD, lung
tissue is damaged, alveoli are destroyed and airways can get irritated
and inflamed (swollen)
How quickly does pulmonary fibrosis progress?
Each year as the disease progresses, pulmonary fibrosis
patients will lose an average of between 150 mL and 200
mL of lung capacity, which can be monitored by spirometry
(breathing tests).
What are the complications of pulmonary fibrosis?
heart failure, stroke, pulmonary embolism, and lung infections.
Weakness in the lungs means that other tissues aren’t getting the oxygen
that they need, and scar tissue makes the lungs more susceptible to
infection.
symptoms of pulmonary fibrosis?
Dry cough that doesn’t go away.
Fatigue (extreme tiredness, no matter how much you sleep).
Shortness of breath, especially during or soon after you exercise.
Weight loss
As the disease progresses, some people experience:
Clubbing, fingertips or toes that look different, such as wider or more round.
Cyanosis, bluish skin (in fair-skinned people) or
gray or white skin around the mouth or eyes (in dark-skinned people)
from too little oxygen in the blood.
.
Diagnosis
• Blood tests: Your provider may order blood tests to rule out other illnesses or reasons
for your symptoms. Lab tests can also help providers track disease progression (how it
affects your body over time) after diagnosis.
• Imaging tests: A chest X-ray or a CT scan may help rule out other lung-related illnesses.
These pictures can clearly show lung scarring and may confirm a pulmonary fibrosis
diagnosis.
• Breathing tests: These tests are also called pulmonary function tests. Different devices
measure lung function and capacity (how well your lungs work).
• Oxygen desaturation study: This test measures oxygen levels in your blood. You walk
for six minutes with a probe attached to your finger or forehead.
• Biopsy: A surgeon removes a small lung tissue sample through a small incision in the
ribs. Providers sometimes perform a lung biopsy to confirm a pulmonary fibrosis
diagnosis.
Treatment
• Most pulmonary fibrosis treatments focus on easing symptoms and improving your quality
of life.
• Your provider may recommend one or more treatments:
• Medication: Two medications — pirfenidone and nintedanib—may slow down lung
scarring. These medications can help preserve lung function.
• Oxygen therapy: Giving your body extra oxygen helps you breathe more easily. It may also
increase your energy and strength.
• Pulmonary rehabilitation: Staying active in this special exercise program may improve
how much (or how easily) you can do everyday tasks or activities.
• Lung transplant: A lung transplant replaces one or both diseased lungs with a healthy lung
(or lungs) from a donor. It offers the potential to improve your health and quality of life. A
lung transplant is major surgery, and not everyone is a candidate. Ask your provider if you
may be eligible for a lung transplant
• What is the life expectancy for people who have pulmonary fibrosis?
• Some people live only months after a pulmonary fibrosis diagnosis. Others live several
years. Many factors affect your prognosis. Even your provider can’t predict some of these
factors.
• If you’ve been diagnosed with pulmonary fibrosis, you can take steps to help your body
stay in its best possible shape:
• Be proactive to avoid getting sick: Pay close attention to handwashing, and avoid direct
contact with anyone you know is sick. Having pulmonary fibrosis means your body can’t
recover as easily from infections that other people may fight off with little effort.
• Keep up-to-date with vaccines: Lung scarring makes it harder for the body to fight
against infections. Get vaccinated for pneumonia and influenza (flu) to minimize your risk.
• Practice healthy habits: Stay active and make smart food choices. Get plenty of rest, and
quit smoking.
