Interstitial Lung Disease
What are interstitial lung diseases?
Interstitial lung disease is the
name for a group of more than 200 ongoing (chronic) lung disorders. These diseases
inflame or scar the lungs. The inflammation and scarring make it hard to get enough
oxygen. The scarring is called pulmonary fibrosis.
The symptoms and course of these diseases may vary from person to person. The common link between the many forms of the disease is that they all begin with an inflammation.
- Bronchiolitis. This is inflammation of the small airways (bronchioles).
- Alveolitis. This is inflammation of
the air sacs where oxygen and carbon dioxide exchange in the blood takes place
- Vasculitis. This is inflammation that
affects the small blood vessels (capillaries).
The most common types of interstitial lung disease are:
- A drug-induced disease
- Exposure to substances at work or in the environment
- Lung injury because of radiation
Fibrosis leads to long-term
(permanent) loss of your lung tissue’s ability to carry oxygen. When the scar tissue
forms, it destroys the air sacs. It also destroys the lung tissue around the air sacs
and the lung capillaries.
The disease may run a slow course
or a rapid course. People that have it may notice variation in symptoms, from very mild
to moderate to very severe. The condition may stay the same for a long time. Or it may
change quickly. The course of the disease is unpredictable. If it progresses, the lung
tissue thickens and becomes stiff. Breathing becomes more difficult.
What causes interstitial lung diseases?
The cause of interstitial lung disease is not known. Major contributing factors are smoking and inhaling environmental or occupational pollutants, such as inorganic or organic dusts.
Other contributing factors include:
- Certain drugs or medicines
- Certain connective tissue or collagen diseases and sarcoidosis
- Family history
- Radiation treatment
What are the symptoms of interstitial lung diseases?
Symptoms are a bit different for each person. Here are the most
- Shortness of breath, especially with
- Dry, hacking cough that does not
- Extreme tiredness and weakness
- No appetite
- Unexplained weight loss
- Mild pain in the chest
- Labored breathing, which may be fast
- Bleeding in the lungs
The symptoms of interstitial lung
diseases may look like other lung conditions or health problems. Talk with your
healthcare provider for a diagnosis.
How are interstitial lungs diseases diagnosed?
In addition to a complete health
history and physical exam, the healthcare provider may also request pulmonary function
tests. These tests help to measure the lungs’ ability to move air into and out of the
lungs. The tests are often done with machines into which you breathe. They may include
A spirometer is a device used to check lung function. Spirometry is one of the simplest, most common tests. It may be used to:
- Determine how well the lungs receive, hold, and move air
- Look for lung disease
- See how well treatment is working
- Determine the severity of a lung disease
- Find out is the lung disease is
restrictive (decreased airflow) or obstructive (disruption of airflow)
Peak flow monitoring
This device is used to measure
how fast you can blow air out of the lungs. Disease-related changes can cause the
large airways in the lungs to slowly narrow. This will slow the speed of air leaving
the lungs. This measurement is very important in evaluating how well or how poorly
the disease is being controlled.
This test takes pictures of internal tissues, bones, and organs.
Arterial blood gas may be done to check the amount of carbon dioxide and oxygen in the blood. Other blood tests may be used to look for possible infections.
This test uses a combination of
X-rays and a computer to make images of the body. CT scans are more detailed than
This is direct exam of the main airways of the lungs (bronchi) using a flexible tube called a bronchoscope. Bronchoscopy helps to evaluate and diagnose lung problems, check blockages, take out samples of tissue or fluid, and help remove a foreign body. Bronchoscopy may include a biopsy or bronchoalveolar lavage.
This test removes cells from the
lower respiratory tract to help find inflammation and rule out certain causes.
This test removes a small piece
of tissue from the lung so it can be checked under a microscope.
How are interstitial lung diseases treated?
Because there are so many causes,
treatment will vary. Some interstitial lung diseases don't have a cure. Treatment is
aimed at preventing more lung scarring, managing symptoms, and helping you stay active
and healthy. Treatment can’t fix lung scarring that has already occurred.
Treatments may include:
- Lung transplant
- Medicine taken by mouth (oral),
including corticosteroids to reduce inflammation and cyclophosphamide to suppress the
- Oxygen therapy, from portable
- Pulmonary rehab
Check with your healthcare provider
about getting flu and pneumococcal shots. Getting a flu shot every year can help prevent
both the flu and pneumonia. In addition, pneumococcal bacteria can cause minor problems
such as ear infections. But they can also develop into life-threatening illnesses of the
lungs (pneumonia), the covering of the brain and spinal cord (meningitis), and the blood
(bacteremia). Anyone can get pneumococcal disease. But those at highest risk include
children younger than age 2, adults ages 65 and older, people with certain health
problems, and smokers.
Talk with your healthcare provider and your child’s provider about
the pneumococcal vaccine. The CDC recommends it for all children younger than 2 years
old and all adults age 65 or older.
Key points about interstitial lung diseases
- Interstitial lung disease is the name
for a group of more than 200 lung disorders that inflame or scar the lungs.
- The cause is often not known. Major
contributing factors are smoking and inhaling environmental or occupational
- The most common symptoms are shortness of breath, especially with activity, and a dry, hacking cough.
- X-rays and other imaging studies are
used to diagnose the condition. So are tests that help measure the lungs’ ability to
exchange oxygen and carbon dioxide.
- The goal of treatment is to prevent
more scarring, manage symptoms, and help you stay active and healthy.
Tips to help you get the most from a visit to your healthcare provider:
- Know the reason for your visit and what you want to happen.
- Before your visit, write down questions you want answered.
- Bring someone with you to help you ask questions and remember what your healthcare provider tells you.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.
- Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
- Ask if your condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if you do not take the medicine or have the test or procedure.
- If you have a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your healthcare provider if you have questions.
Online Medical Reviewer:
Alan J Blaivas DO
Online Medical Reviewer:
Daphne Pierce-Smith RN MSN CCRC
Online Medical Reviewer:
Marianne Fraser MSN RN
Date Last Reviewed:
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