A B C D E F G H I J K L M N O P R S T U V W

FIBROSING ALVEOLITIS

What is Fibrosing Alveolitis ?

The lungs are found in the chest, on either side of the heart, beneath the protective rib cage. They are made up of a series of branching tubes (the bronchi) leading all the way down from the main windpipe (trachea) to the system of air sacs (alveoli). This is where the exchange of gases takes place. In the alveoli oxygen is absorbed from the breathed air, through their walls into the bloodstream. Normally the lungs are able to expand and shrink with breathing, enabling air to be drawn in and out. Fibrosing alveolitis (FA) is a condition in which these walls of the lung tissues become thickened. This thickening impairs the ability of the lungs to expand, with the result that less air can be breathed in. This results in less absorption of oxygen into the bloodstream and leads to breathlessness. The thickening is due to scarring (fibrosis), plus the presence of large cells and inflammation. The main symptom of FA is increasing breathlessness. Coughing may occur at some stages of the illness, but is not a striking feature.

How does Fibrosing Alveolitis occur ?

The cause of this condition is still not known. It is probably the result of overactivity of the body's immune system causing damage to the lungs. The trigger for this self-damaging (auto-immune) process is not known, but may be multiple.

Why does Fibrosing Alveolitis occur ?

Very rarely fibrosing alveolitis runs in families, but no clear genetic inheritance has been demonstrated. The disease is sometimes associated with other conditions, which are also auto-immune in origin e.g. Rheumatoid arthritis. A condition very similar to FA may be caused by certain drugs e.g. cancer chemotherapy drugs busulphan, bleomycin; and poisoning with the weedkiller paraquat may produce a disease very like a severe form of FA.

Treatment Involved for Fibrosing Alveolitis

Diagnosis is best made by a CT (computerised tomography) scan, which is a special type of Xray and lung function tests. In many patients the disease progresses very slowly, or may even remain static. Under these circumstances, treatment may not be required, and you may simply be kept under observation at a chest clinic. However, when the disease is progressing, treatment is required. Usually this will involve steroid (cortisone-like) tablets and these may need to be taken in high dosage. The steroids may be used in combination with, or changed to, other immunosuppressant drugs. Such drugs might include cyclophosphamide or azathioprine. Steroid drugs must always be taken as prescribed. The dose tends to be high at the beginning of treatment, in order to bring about a response. The dose is then lowered steadily to a maintenance level, but it may need to continue at this level for life. Immunosuppressant drugs again must be taken regularly as prescribed. The doctor will need to check your blood count regularly, especially at the start of treatment. Too high a dose may suppress normal blood cell production in the bone marrow and lead to problems.

During Treatment for Fibrosing Alveolitis

Just as the onset of the disease is usually slow, improvement also takes time. It may be that progress of the disease is stopped by treatment, resulting in a period of stability. Steroid drugs may cause indigestion, fluid swelling, weight gain, and a tendency to bruise easily. They can even trigger diabetes, with marked thirst and frequent urination. If they occur, these side effects should be reported, since the dosage or timing of the drugs may be changed to minimize them. Immunosuppressant drugs may alter the ability to fight infections. Any infection should be reported to the doctor immediately, as blood tests may need to be checked and the dosage of the drugs altered. Antibiotics will probably be needed for any infection, and the dose of steroids may need to be increased for a time. If there is no response to treatment, the specialist may wish to try other drugs. If despite this breathing deteriorates, oxygen may need to be provided in the home.

If Fibrosing Alveolitis is Left Untreated

The disease might remain stable, but in most patients it would progress and, ultimately, prove fatal. Increasing breathlessness would occur, and any minor infections in the lungs would become very difficult to cope with.

Effects on Family of Fibrosing Alveolitis

The chance of the condition being inherited is so small that routine testing is not necessary. Carers may need to be taught how to help in the use of oxygen at home, and occasionally in physiotherapy. They should appreciate the need for extra time for the patient to carry out activities in order to avoid breathlessness.