DEFINITION OF
INTERSTITIAL LUNG DISEASE
Interstitial Lung Disease (ILD); this is a term which includes a variety of
chronic lung disorders. In a person suffering from ILD, the lungs are affected
in three ways.
First, the lung tissue is
damaged in some known or unknown way.
Second, the walls of the
air sacs in the lung become inflamed.
Finally, scarring (or
fibrosis) begins in the interstitium (or tissue between the air sacs), and the
lung becomes stiff.
Breathlessness
induced by physical stress can be one of the first signs of these diseases. Dry
cough could also be present. These are common symptoms that many people ignore.
People
with different types of ILD may have the same kind of symptoms but their
symptoms may vary in severity even though their
chest X-rays may look alike. Further testing is usually recommended to identify
the specific type of ILD a person has.
Some
ILDs have known causes and some (idiopathic) have unknown causes.
WHY THE NAME?
The
tissue between the air sacs of the lungs is called the interstitium and as Interstitial
lung disease affects this tissue hence the name. The tissue is affected by
fibrosis (scarring). Interstitial lung disease is sometimes also known as
"interstitial pulmonary fibrosis." The terms interstitial lung
disease, pulmonary fibrosis and interstitial pulmonary fibrosis are also used for
the same condition.
INTERSTITIAL LUNG DISEASES ARE PUZZLING
The course of this disease is
unpredictable. If they progress, the lung tissue thickens and becomes stiff. Breathing
then becomes difficult and demanding. This may improve with medication if
treated when inflammation occurs. In certain cases oxygen therapy may be needed
as part of the treatment.
The diseases may have a
gradual course or a rapid course. The symptoms may vary from very mild to
moderate to very severe. Their condition may remain the same for long durations
or may change quickly.
POSSIBLE LINKS IN
INTERSTITIAL LUNG DISEASE
Eventhough the progress
and symptoms of these diseases may vary from person to person, there is usually
one common link between the different forms of ILD and that is their begin with
an inflammation. The inflammation therefore may affect different parts of the
lung, as may be explained below:
Bronchiolitis, Alveolitis, or Vasculitis
.
The inflammation of these
parts of the lung may heal or may lead to permanent scarring of the lung
tissue. When scarring of the lung tissue takes place, the condition is called
pulmonary fibrosis.
Fibrosis of the lung
tissue, results in permanent loss of that tissue's ability to transport oxygen.
The level of disability depends on the amount of scarring of the tissue. This
is because the air sacs, as well as the lung tissue between and surrounding the
air sacs, and the lung capillaries, are destroyed by the formation of scar
tissue.
WHAT ARE THE KNOWN
CAUSES OF PULMONARY FIBROSIS?
Several causes of pulmonary fibrosis are known. They include:
1.
Occupational and environmental exposures. Exposure to metals, dust and pollen.
2. Sarcoidosis.
3. Drugs.
4. Radiation.
4. Connective tissue
or collagen diseases
5. Genetic/familial.
IDIOPATHIC PULMONARY FIBROSIS (IPF)
when we do not have a known cause for this then we term it as "idiopathic"
(of unknown origin) pulmonary fibrosis (IPF).
THE
SYMPTOMS OF IPF
Shortness of breath - the main symptom of idiopathic pulmonary fibrosis.
Other symptoms may
include a dry cough (without sputum). When the disease is severe and prolonged,
heart failure with swelling of the legs may occur.
DIAGNOSIS
OF IPF
A very careful patient history is an important tool for diagnosis. The history
will includes environmental and occupational factors, hobbies, legal and
illegal drug use, arthritis, and risk factors for diseases that affect the
immune system. A physical examination, chest X-ray, pulmonary function tests,
and blood tests are important..
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Diagnostic Tests That May Be Used to Identify Pulmonary Fibrosis or
Interstitial Lung Disease
1. Blood Tests
2. Pulmonary Function Tests
3. Chest X-ray
4. CT Scan
5. Bronchoscopy
6. Bronchoalveolar Lavage
7. Lung Biopsy
8. CT Scan
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TREATMENT FOR IPF
Corticosteroids may be given to treat the inflammation.Other drugs are occasionally
added when it is clear that the steroids are not effective in reversing the
disease.
Oxygen therapy may be
prescribed for some people with IPF. The need for oxygen will be determined by
your doctor and may depend on the severity of disease, as well as your activity
level. Talk with you doctor if you think you may need oxygen or if you have
concerns about oxygen.
Influenza vaccine and
pneumococcal pneumonia vaccine are both recommended for people with IPF or any
lung disease.
Rehabilitation and
education programs may help some people with IPF. Local support groups have
been of benefit for people with IPF and their family members and friends.
