The
interstitial lung diseases (ILD) are a varied group of
conditions that mainly affect the lung tissue itself
rather than the airways (breathing tubes). They cause
both inflammation and scarring of the lung, the latter
also known as fibrosis. These diseases are often first
picked up on a chest x-ray, perhaps performed because of
shortness of breath or a troublesome dry cough.
Occasionally the diagnosis is made because patients have
manifestations of other, more generalised conditions
such as sarcoidosis, rheumatoid arthritis or a connective
tissue disease.
Patients referred for investigation of possible ILD will
typically have initial investigations that include blood
tests and a CT scan, to give clues to the cause of lung
scarring, and breathing tests to assess the extent to
which the function of their lungs is being affected. In
order to make a definite diagnosis of the cause, we may
have to perform additional investigations such as
bronchoscopy and sometimes a surgical lung biopsy. These
investigations are important in reaching a diagnosis,
making treatment decisions and also in giving patients
information about their prognosis.
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The
commonest interstitial lung disease (ILD) is fibrosing
alveolitis, sometimes called “cryptogenic” because its
cause is unknown, and also called idiopathic pulmonary
fibrosis. An apparently identical condition also occurs
as a complication of rheumatoid arthritis. A similar
condition can be found in connective tissue diseases,
particularly scleroderma. There are approximately 2,500
new diagnoses each year so that, although not rare,
patients tend to be referred to clinicians who have an
interest n this disease and who are used to treating it.
Fibrosing alveolitis is difficult to treat although a
number of newer drugs are becoming available and some
are entering clinical trials soon. Further information
on
this condition is available from the
British Lung Foundation
and from
European Lung Foundation.
The other common ILD is a condition called sarcoidosis.
Again the cause is unknown but it can affect many
different parts of the body including the skin, lymph
glands and sometimes the lung. In general, sarcoidosis
can be treated successfully especially if diagnosed
early but a small number of patients do develop
significant lung scarring. Again, more information can
be obtained from
The British Lung Foundation.
There are many rarer forms of ILD. In the ILD Clinic the
doctors and respiratory nurse specialists will give
patients full information about their condition to help
then to understand what it means and explain the
rationale for their investigations and treatment. |
