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

POLYARTERITIS - SCLERODERMA - POLYMYOSITIS

What is Polyarteritis ?

Connective tissue disease is the name for a group of conditions which affect many organs or tissues in a particular way. There is widespread inflammation. The disorders have many symptoms in common. All patients have the features of general inflammation : fever, weight loss, tiredness and anaemia. Most patients will also have inflammation of joints, producing a type of arthritis. The joints are red, hot, swollen, stiff and painful. Inflammation of the small blood vessels (called vasculitis) is also common in this group of conditions. Vasculitis causes swelling in the walls of tiny blood vessels, resulting in a blocking of the blood supply to that tissue or organ. If the circulation to the hands is affected, the fingers will go white and then blue when exposed to cold (Raynaud’s phenomenon). Involvement of the kidney may result in high blood pressure. As the inflammation settles, all symptoms improve. However, if the process continues for a long time, scarring occurs in the affected blood vessels and the tissue supplied by those blood vessels will then be damaged permanently. As well as the symptoms common to the whole group, each connective tissue disease has its own particular symptoms. To complicate matters further, an individual may have symptoms from different disorders. This is then called MIXED CONNECTIVE TISSUE DISEASE. In SCLERODERMA, there is excessive thickenining of tissues. The skin is hard, and loses its ability to stretch. The fingertips are thickened and the skin around the mouth tightens up. In POLYMYOSITIS, the inflammation is mainly in the muscles: pain and weakness are the main symptoms. POLYARTERITIS NODOSA produces widespread inflammation in blood vessels. Any tissue can be affected. In the skin, rashes occur, while in the lungs it produces a type of asthma, and cavities can develop. In the nervous system it can cause strokes, with paralysis of limbs and other nerve symptoms. All these connective tissue diseases are rare.

How does Polyarteritis occur ?

There is good evidence that this group of diseases have a common cause, although its exact nature is unknown. However, it is likely to be a defect in the immune system. The immune (defence) system of the body is normally triggered by a foreign agent such as a virus. Certain white cells in the blood (lymphocytes) and chemicals called antibodies are automatically sent to destroy this agent. In connective tissue diseases these lymphocytes and antibodies (auto-antibodies) over-react. Not only do they destroy the foreign agent, but they also damage the body's own tissues. The inflammation leads to scarring and tissue damage which is so typical of this group of disorders.

Why does Polyarteritis occur ?

Inherited factors seem to be unimportant. It is not known why it suddenly starts or why individuals have different forms of connective tissue disease. They all commonly start in young adults.

Treatment Involved for Polyarteritis

Treatment is aimed either at suppressing the abnormal antibodies or white blood cells (the immune system) in your body, or reducing the intensity of the inflammation. The drugs used need careful supervision by hospital specialists. Steroids are cortisone-like drugs which reduce inflammation. They are given as tablets in high doses at the start, and then reduced as the inflammation settles. Disease-modifying drugs act on the immune system. Response to them is rather unpredictable. These drugs, amongst which are methotrexate, azathioprine and cyclophosphamide, are used in polymyositis and polyarteritis nodosa. They may be given as tablets or directly into a vein during your admission to hospital. All disease-modifying drugs need careful supervision by hospital specialists with blood tests, which help to regulate the doses used. Scleroderma does not consistently respond to any drug, but many new ones are being tried in specialist centres. Treatment of individual symptoms is very important in these diseases. Painkillers and anti-inflammatory tablets are used. Special drugs may also help the Raynaud's phenomenon, as may special heated gloves to keep the hands warm. Physiotherapy has an important role in preventing joint deformities, especially in polymyositis.

During Treatment for Polyarteritis

Steroids may cause you to gain weight with swelling of the face and more fat around your middle. Your arms and legs tend to get thin. The skin may bruise easily and fluid retention may result in swollen ankles. Diabetes and high blood pressure may develop. All these changes may be reversible on stopping the drug. If taken for a long period, steroids tend to thin the bones (osteoporosis)and make them more liable to fracture. Disease-modifying drugs can cause rashes and some loss of hair. The hospital should be contacted at any time that you notice any possible side-effects, as the drugs may need to be stopped staight away.

After Treatment for Polyarteritis

The connective tissue diseases tend to have a long course lasting many years. The effect on the individual varies with the system or tissue affected. If you have kidney involvement there is a tendency to have more severe disease.

If Polyarteritis is Left Untreated

This depends on the disorder. In polymyositis, steroids are often life saving. In polyarteritis nodosa and severe vasculitis the outcome is poor without treatment.

Effects on Family of Polyarteritis

With this group of chronic diseases you will need a lot of support to help you cope. You will need to attend hospital regularly and will need hospitalization from time to time. Your family and friends can help you deal with these stresses.