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Lupus and Associated Conditions


Hughes' syndrome (antiphospholipid syndrome)

This is often called "sticky blood" in that patients have a tendency to clots, both in the veins and arteries. The thrombosis may present either dramatically, or over a period of time with clots in major vessels, even including the brain. It is vital that this diagnosis is made (a simple blood test for antiphospholipid antibodies is available in most major hospitals). Pregnant women who have antiphospholipid antibodies have a tendency to clot in the placenta and develop recurrent miscarriages and these can be prevented by diagnosis and treatment. Although this syndrome was first discovered in lupus patients it is now recognised that many, if not the majority of patients with Hughes' syndrome, have no other features of lupus nor will they develop lupus in the future.

Mixed Connective Tissue Disease

The name "mixed" was applied because patients with this syndrome have features in common with two or three diagnoses - lupus, scleroderma and myositis (muscle inflammation). The predominant features of this syndrome are very severe Raynaud's Phenomenon (cold, blue fingers) and joint pains, often with puffy, swollen "sausage" fingers.

What parts of the body does lupus usually affect?

Systemic lupus erythematosus (SLE or lupus) is one of the so-called connective tissue disorders
that are caused by a fault in the body's immune system, which normally fights infection. In this condition, antibodies are made, which instead of killing germs, attack the body itself and cause damage.
Lupus can vary from a very mild disease for which no treatment is required to a much more serious condition which needs very strong medication. Most people lie somewhere between these extremes. A rash is a very common problem often made worse by the sun (photosensitivity). Mild hair loss (which comes and goes) can be a feature of the disease. Joint pains, particularly in the hands and feet, can also be a problem as can general tiredness. Sometimes the circulation into the fingers can be affected and this causes them to go white and numb in cold weather. As the fingers recover they turn blue then red. This is called Raynaud's Phenomenon. Lupus can affect many other parts of the body including the kidneys, brain, nerves and lungs. This is much less common. However, when lupus does this it often requires very powerful medication. Kidney problems can also cause high blood pressure.

Are people with lupus more likely to get other problems where the immune system attacks the body?

The answer to this is yes. Anyone with one condition where the immune defence mechanism attacks the body (so-called autoimmunity) is prone to another one. We know this is the situation with all autoimmune diseases. For example, people with diabetes (where the body attacks its insulin making cells) are more likely than the general population to get rheumatoid arthritis or thyroid diseases. In general, if a patient has lupus combined with another autoimmune disease, the lupus itself tends to be less severe. In the same way, the other autoimmunune disease, which can occur without lupus, tends to be milder. Correspondingly, doctors can get away with less powerful treatment in these 'overlap' or 'mixed connective tissue' disorders.

What are the other problems that people with lupus are prone to?

One of the conditions that can occur along with lupus is Sjögren's syndrome. This can occur in about 1 in 5 patients with lupus. With this problem the immune system attacks the glands that produce fluids to lubricate different parts of the body. Therefore dry eyes and dry mouth can be a problem. The doctor can provide artificial tears or saliva to help with this. There are some special fruit pastilles that can be sucked to help the production of saliva. However, it is important that these are sugar-free as the lack of saliva increases the risk of tooth decay. There is a blood test for a specific antibody that goes with Sjögren's syndrome. Sometimes a tiny piece of tissue can be removed from the lower lip to detect the condition (a lip salivary gland biopsy).

Sjögren's syndrome can also cause dryness of the vagina. This can make sexual intercourse uncomfortable. There are lubricants available to help with this too.

A little under 1 in 10 patients with lupus suffer with another problem of autoimmununity. This is where the body attacks the thyroid gland that controls the body's metabolism. The thyroid gland can either become underactive or overactive. If the gland is overactive, it can cause sweating, anxiety, shaking, heart pounding and weight loss. If underactive, the skin becomes dry, weight gain is a problem and mental alertness can be affected. Sometimes the thyroid gland (which is found at the front of the neck) can become swollen. A thyroid problem is easily detected with a simple blood test. It is also quite easily treated.

In lupus, as discussed above, joint pain is a common problem but this pain is not associated with actual damage to the joint itself. Much less often, people with lupus can develop arthritis where the joints are affected in the same way as is found in rheumatoid arthritis. Therefore lupus and rheumatoid arthritis can happen in the same person. In rheumatoid arthritis, there is swelling of the lining of the joints. This swollen lining is called the synovium. Normally it is very thin and stops the natural fluid lubrication of the joint from leaking out. However, when inflamed it becomes very swollen, red and angry. It can eat its way into the bones and cause damage to them. Doctors use tablets in this situation to reduce the swelling of the synovium. By doing this they hope to reduce or halt the damage that can happen. The choice of tablets for this when lupus overlaps with rheumatoid arthritis are very similar to those used in rheumatoid arthritis alone.

The muscles of the body can also become inflamed in lupus. Again this can happen in patients without lupus and the treatments are very similar. The muscles can become very painful and weak if this is a problem. There are special blood tests and muscle electrical tests used to look for damage to the muscles from inflammation (myositis). Sometimes a small piece of muscle needs to be removed to look for the problem under the microscope (muscle biopsy).

Another problem with the immune system that can occur with lupus is thickening of the skin which causes it to become light and hard - particularly over the fingers and face. This is called scleroderma and is very rare. People with this condition are very prone to Raynaud's. It can also cause the thickening of other tissues, which can cause difficulty in swallowing and diarrhoea. There are simple treatments available to help with the swallowing and diarrhoea but skin thickening is very difficult to treat and tends to be permanent. Patients with scleroderma without lupus are prone to scarring of the lung and involvement of the kidneys in the same way as is found in lupus itself.

Other diseases

Other conditions occasionally wrongly diagnosed as lupus include Wegener's granuloma (antiinflammatory disease of the sinuses and chest totally different from lupus in fact), fibromyalgia (commonly diagnosed in early stages of some lupus patients) and multiple sclerosis.





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