What Is Pulmonary Fibrosis?
Pulmonary fibrosis is a condition
that causes lung scarring and stiffness. This keeps the body from getting
enough oxygen. Increased scarring makes breathing difficult and affects the
What Causes Pulmonary Fibrosis?
The causes of pulmonary fibrosis
can be divided into four categories: autoimmune diseases, genetics, chemical exposure,
and unknown causes.
Autoimmune diseases cause the
body’s immune system to attack itself. Conditions that can result in pulmonary
- Churg-Strauss syndrome
- lupus erythematosus (lupus)
- rheumatoid arthritis (RA)
Lupus, RA, and scleroderma are
most commonly associated with pulmonary fibrosis (Pulmonary Fibrosis
Exposure to altering agents can cause
pulmonary fibrosis. Cigarette smoke contains many chemicals that can damage the
lungs. Other substances that damage the lungs include asbestos fibers, gases,
grain dust, radiation, and silica dust.
Some medications can be
associated with pulmonary fibrosis. Patients with a family history of pulmonary
fibrosis should speak with their doctors before accepting certain medications. Some
medications that can damage the lungs include:
- cardiac drugs, such as amiodarone or propranolol
- chemotherapy drugs, such as cyclophosphamide
- select antibiotics, such as nitrofurantoin or
Some causes of pulmonary fibrosis
are unknown. When this is the case, the condition is called idiopathic
pulmonary fibrosis (IPF). This diagnosis represents a significant portion of
pulmonary fibrosis cases (National
Heart, Lung and Blood Institute, 2011).
Who Is at Risk for Pulmonary Fibrosis?
For the most part, pulmonary
fibrosis does not have a known cause.
People with a greater likelihood
of being diagnosed with pulmonary fibrosis:
- are between the ages of 40 and 70
- are male (Pulmonary Fibrosis
- have a history of smoking
- have undergone cancer treatments, particularly
- work in an occupation associated with increased
risk, such as mining, farming, or construction
A genetic counselor can evaluate
your health risks if you have a pulmonary fibrosis family history.
What Are the Symptoms of Pulmonary Fibrosis?
A person can have pulmonary
fibrosis for some time without any symptoms. Shortness of breath is typically
the first symptom. Because the condition generally affects older people, symptoms
are usually attributed to age or lack of exercise.
Pulmonary fibrosis affects the lungs
and breathing. Symptoms include:
- abnormal lung sounds, such as crackling
- aching joints and muscles
- a chronic, dry, and hacking cough
- clubbing, where fingernails appear curved
- frequent shortness of breath
- sudden weight loss
Symptoms may seem minor at first
and progress over time. But symptoms can vary from person to person. Some
patients become ill very quickly.
How Is Pulmonary Fibrosis Diagnosed?
More than 200 types of lung disease exist. Pulmonary
fibrosis is one of them. Because there are so many, doctors often have trouble
identifying IPF over other conditions. As many as 50 percent of patients with
pulmonary fibrosis may have been misdiagnosed at one time (Pulmonary Fibrosis
A doctor will use a variety of
diagnostic tools to diagnose pulmonary fibrosis. These include imaging scans,
such as a chest X-ray, echocardiogram, and computed tomography (CT) scan.
Other tests measure how well the lungs
are working. A spirometry can test lung capacity. A treadmill stress test shows
how lungs respond to physical activity.
The most definitive way to
diagnose pulmonary fibrosis is by taking a tissue sample. This is an invasive
procedure. A tissue sample can be taken with a bronchoscopy, in which a small
tube is passed through the mouth to the lungs.
If a larger sample is needed, a
surgical biopsy is performed. A physician makes several small incisions to
access the lung tissue. A physician reading the biopsy can decide how far the condition
How Is Pulmonary Fibrosis Treated?
There is no cure for pulmonary fibrosis. Scarring cannot be
reversed. Treatments aim to slow the disease and improve breathing. The best
treatments and medications vary by individual patient.
Medications used to treat pulmonary fibrosis have different
aims. Examples include:
- Prednisone is a corticosteroid that lowers the
immune system and reduces inflammation.
- Methotrexate and cyclosporine suppress the
- N-acetylcysteine is an antioxidant often used
with other drugs to slow the disease’s progression.
- Pirfenidone reduces inflammation and scarring; this
medication is pending U.S. approval as of April 2013 (Pulmonary Fibrosis Foundation,
Oxygen therapy can make breathing
and sleeping easier. This therapy involves wearing a plastic tube in each nostril.
The tubes receive oxygen from a tank. Pulmonary rehabilitation is another
option. It focuses on techniques that make breathing easier.
Younger patients who do not
respond to these treatments might need a lung transplant.
Smokers with pulmonary fibrosis
should take steps to quit smoking. This slows the disease’s progress and eases
breathing. Patients should also avoid secondhand smoke.
People with pulmonary fibrosis often
lose weight because eating affects breathing. A nutritionist or dietitian can
recommend high-calorie foods that are easy to eat.
Lifestyle changes, including exercise,
improve lung function and strengthen the heart. Stronger muscles mean a patient
becomes tired less easily and has more energy. Getting lots of rest and trying
to stay relaxed whenever possible can improve breathing.
What Is the Outlook for Pulmonary Fibrosis?
The rate at which pulmonary fibrosis scars the lungs varies
from person to person. Scarring cannot be reversed. A doctor can try different
treatments to reduce the rate at which pulmonary fibrosis progresses. People
diagnosed with pulmonary fibrosis typically live about three to five years (American Lung
The condition causes a number of complications, including
lung cancer. It can also cause respiratory failure. This is when the lungs no
longer work properly and cannot get oxygen to the blood.
How Can Pulmonary Fibrosis Be Prevented?
Causes of IPF are unknown, so prevention is not possible.
The best way to prevent pulmonary fibrosis is to avoid smoking and secondhand
smoke and thus limit exposure to harmful chemicals. Factory workers should wear
proper breathing devices to limit exposure.