The Division of Pediatric Pulmonary Medicine at MassGeneral Hospital for Children is a well-established clinical, training and research program. The group provides multidisciplinary comprehensive consultation, diagnostic and management services for a wide array of pulmonary conditions.
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The Massachusetts General Hospital Interstitial Lung Disease Program provides the latest diagnostic and therapeutic options for patients with the roughly 160 disorders categorized as interstitial lung diseases, also known as pulmonary fibrosis.
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The Lung Transplant Program at the Massachusetts General Hospital Transplant Center brings together state-of-the-art technology and leading-edge medical and surgical interventions to provide patients with individualized care before and after their lung transplants.Download our patient guide to transplantation (PDF) Ver información e indicaciones para el paciente en español (PDF)
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Interstitial Lung Disease
What are interstitial lung diseases?
Interstitial lung disease is the name for a group of 100 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 places (alveoli).
- Vasculitis. This is inflammation that involves 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 due to radiation
Fibrosis leads to permanent loss of your lung tissue’s ability to carry oxygen. The air sacs, as well as the lung tissue around the air sacs and the lung capillaries, are destroyed when the scar tissue forms.
The disease may run a gradual 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?
The following are the most common symptoms for interstitial lung diseases. However, each person may experience symptoms differently. Symptoms may include:
- Shortness of breath, especially with activity
- Dry, hacking cough that does not produce phlegm
- Extreme tiredness and weakness
- Loss of appetite
- Unexplained weight loss
- Discomfort in the chest
- Labored breathing, which may be fast and shallow
- Bleeding in the lungs
The symptoms of interstitial lung diseases may look like other lung conditions or medical problems. Talk with your healthcare provider for a diagnosis.
How are interstitial lungs diseases diagnosed?
In addition to a complete medical 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 usually done with machines into which you breathe. They may include the following:
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 whether the lung disease is restrictive (decreased airflow) or obstructive (disruption of airflow)
Peak flow monitoring
This device is used to measure the 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 computer technology to produce horizontal, or axial, images (often called slices) of the body. CT scans are more detailed than regular X-rays.
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.
Removing cells from the lower respiratory tract to help identify inflammation and exclude certain causes.
Removing a small piece of tissue from the lung so it can be examined under a microscope.
How are interstitial lung diseases treated?
Because there are so many causes, treatment will vary. Some interstitial lung diseases do not 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
- Oral medicine, including corticosteroids to reduce inflammation and cyclophosphamide (Cytoxan) to suppress the immune system
- Oxygen therapy, from portable containers
- 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 can also develop into life-threatening illnesses of the lungs (pneumonia), the covering of the brain and spinal cord (meningitis), and the blood (bacteremia). Pneumococcal disease can be acquired by anyone, but children younger than age 2, adults ages 65 and older, people with certain medical problems, and smokers are at the highest risk.
Key points about interstitial lung diseases
- Interstitial lung disease is the name for a group of 100 lung disorders that inflame or scar the lungs.
- The cause is not known. Major contributing factors are smoking and inhaling environmental or occupational pollutants.
- The most common symptoms are shortness of breath, especially with activity, and a dry, hacking cough.
- Tests that help measure the lungs’ ability to exchange oxygen and carbon dioxide are used to diagnose the condition. Blood tests and imaging tests may also be used to see how severe the problem is and monitor it over time.
- The goal of treatment for people with the disease is to prevent more scarring and manage symptoms.
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.