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This is a group of diseases that are partially or totally attributed to immune disorders. Autoimmune disorders occur as a result of a malfunction in the immune system which makes it target specific cells in the host body. The majority of these diseases can only be treated medically as they tend to affect the very small vessels.
Whilst its name suggests inflammation of an artery that runs in the temporal region of the head, this disease can affect any small or medium sized artery in the body. The arteries most affected are those of the upper limbs and head and neck. The disease is two to four times more common in females. Its incidence gradually increases after the age of 50 to reach 5 in 1000 for ages between 70 and 80 years. It starts with an episode of flu-like illness which may be followed few weeks later with persistent headache, shoulder pain, muscular pains and in some cases, partial to complete blindness. It is for this last symptom that early diagnosis and treatment are important. Treatment of temporal arteritis is essentially medical, using variable doses of steroids. Surgery may be required when the main arteries to the arm or head are significantly narrowed.
This is the name given to prolonged contraction and spasm of the arterioles (very small arteries) in the extremities. The primary form occurs in young women and is due to abnormal sensitivity to the cold. The secondary form is seen in collagen (autoimmune) diseases such as scleroderma, lupus and rheumatoid arthritis. Treatment is usually for the underlying condition and advice given to protect from the cold.
This is a disease that almost exclusively affects middle aged smokers. The disease targets the small arteries and veins in the upper and lower limbs. The underlying pathology is an inflammatory process that affects all the layers of the diseased vessel wall. This inflammation subsequently leads to wide spread occlusion of these small vessels. Whilst the exact aetiology of Buerger's disease remains unclear, tobacco (in any form) has a very strong effect on it's initiation and progression. The symptoms of Buerger's disease are those of critical ischemia. The treatment is essentially by stopping smoking and by improving tissue perfusion either pharmacologically or surgically. In those who continue to smoke, 43% will require amputation.
In this type of arterial inflammation, medium to large size arteries are affected. The disease can therefore affect the aorta, renal arteries, innominate, carotid and subclavian arteries. An aneurysmal form of the disease is also known to affect the aorta and its major branches. Takayaso's disease typically affects young females of Asian origin, and is very rare in other races. Patients most commonly present with episodes of visual disturbances, headaches, dizziness, weakness, loss of wrist pulses and high blood pressure. The treatment for Takayasu's disease is mainly medical. Surgery may be required particularly to improve the blood supply to the brain. Takayasu's disease is slowly progressive with a 10 year survival of 90%. Some forms, however, are more aggressive and can significantly reduce the patient's life expectancy. |
Small vessel inflammation leading to painful inflammatory lesions at angles of fingers in a patient with rheumatoid arthritis.
Toe pulp necrosis in the same patient. |
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