FIBROSING ALVEOLITIS
Fibrosing alveolitis is a condition caused by cells that are normally
involved in the body's own defence against infection, instead causing
inflammation, injury, and scarring in the lungs. Scars serve a good purpose in
the skin where they heal injured areas, but in the lung, scar tissue prevents
the lung performing its normal function of taking oxygen from the air into the
blood and removing carbon dioxide from the blood.
Incidence
Fibrosing alveolitis seems to be on the increase although it is not clear
why this is so. It can affect people of any age, but the most common age at
which the disease strikes is in the 50s, and men and women are equally
affected. The cause of fibrosing alveolitis is unknown, but it is not an
infection (and so cannot be caught from others), nor is it a form of cancer.
Exposure to certain occupational dusts (e.g. asbestos, hard metal alloy),
can produce disease which is identical to fibrosing alveolitis, and most people
with the disease are or have been cigarette smokers. However, for most people
the specific cause or provoking factors cannot be identified.
Symptoms
The most common symptom is breathlessness, particularly on exercise such as
walking up hills or stairs. Because it may come on in the mid 50s, patients
often attribute this to middle age. It is important to stress that the onset of
breathlessness should be investigated, rather than to assume it is merely part
of an ageing process. If it remains untreated, the condition can often worsen
and lead to permanent and progressive breathlessness.
Less common symptoms include a dry cough, and some people may notice a
change in the shape of their finger and toe nails. This feature is present in
the majority of patients who develop this lung disease.
Occasionally the first indication of fibrosing alveolitis may be
abnormalities seen on a chest x-ray performed for an unrelated reason.
Diagnosis
Because fibrosing alveolitis is one of the less common lung diseases
(approximately 5 per 100,000 of the population are believed to suffer from
fibrosing alveolitis, although many cases may go undiagnosed) investigations
require a referral to a specialist.
Investigations usually include a chest radiograph and a set of breathing
tests. These require the individual to breathe in and out of a number of
machines which allow an assessment to be made of how well the lungs are
working. Blood tests are also usually performed. A special form of x-ray (known
as a CT scan) which produces a three dimensional picture of the lungs may be
requested.
It may be necessary to obtain samples of lung tissue for examination in the
laboratory. This is usually done by bronchoscopy, which involves passing a
small flexible telescope down the breathing tubes with the patient lightly
sedated. For many people, these tests provide the necessary information for
planning treatment, but in some people a larger sample of lung tissue, taken by
a surgeon under a general anaesthetic, is needed before treatment can be
considered.
Treatment
Fibrosing alveolitis usually requires treatment, even though symptoms appear
to be mild. This is because there is already substantial inflammation in the
lungs by the time symptoms occur. It is very important that the disease is
identified and investigated at the earliest possible stage, so that treatment
to reduce progressive lung scarring can be considered at a time before the
patient is severely disabled. It is widely recognised that treatment is more
effective when it is given before extensive lung scarring has occurred. The
type of treatment recommended depends on the results of the tests outlined
above.
The most common form of treatment is steroids, usually a short period of
high dose therapy followed by a longer period of treatment on lower doses. A
few people may experience side-effects. Therefore a very careful balance
between the potential benefit of treatment and the risk of side-effects needs
to be weighed up before recommendations are made.
Other drugs which may be used to treat the disease are known as
immuno-suppressants. This means that these drugs suppress the body's defence
mechanisms which are believed to be contributing to the disease, but they can
also result in increased susceptibility to infection. People taking these drugs
need to have their blood checked on a regular basis, to make sure that they are
not receiving too much treatment.
Response to treatment is assessed by changes in symptoms, and in the results
of some of the above tests, which may need to be repeated. To achieve this
goal, patients are usually seen on a regular basis. Once maximum response has
been achieved, the aim is to maintain that response while reducing therapy.
Treatment is usually lifelong. This is because the condition is suppressed
rather than cured in the majority of cases, and the long-term aim is to keep
the disease suppressed on the smallest possible dosage of treatment. Very
occasionally, drugs may be discontinued completely, but this is the exception
rather than the rule.
For further help and advice, contact:
British Lung Foundation
8 Peterborough Mews
London
SW6 3BL
 020-7371 7704
(Birmingham): 0121 236 8611 (ext 5985)
(Bristol): 01272 594959
(Glasgow): 0141-204 4110
(Liverpool): 0151-228 4723
(Newcastle): 0191-263 0276
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