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Systemic lupus erythematosus: the great deceiver

Lupus is a chronic disease that usually causes inflammation of the joints, but also attacks the skin, kidneys, blood vessels, nervous system and almost all other organs. Nearly one in 750 women may suffer from lupus. Women are about nine times more likely develop the disease than men. This is mainly a young women's disease and it is even more common among ethnic minorities: in Jamaica it affects one in 250 women.

What is lupus?

Lupus is an autoimmune disease where the immune system produces large quantities of several different types of antibodies that attack the body's own tissues, causing symptoms that strongly resemble those of rheumatoid arthritis.

What is the cause of lupus?

Experts believe that the susceptibility to develop lupus is inherited. It is known to affect monozygotic twins, and the closest relatives of the patients have a much higher probability of developing the disease than others. In lupus, the body produces antibodies that attack the core and other parts of its own cells. A certain event, such as infection or stress can trigger the development of lupus in people with genetic susceptibility. Since lupus is a disease that almost always affects young women, hormones might also play a role in its instigation.

Lupus triggered by drugs

Interestingly, many drugs that are commonly used to treat other conditions may cause temporary lupus, which disappears once the drug concerned is stopped being taken. Hydralazine (drug for high blood pressure), minocycline (used to treat acne), procainamide (used to treat heart rhythm irregularities) and isoniazid (a drug for tuberculosis) are the main culprits. This discovery has attracted the attention of scientists who study how drugs affect the immune system, and it could cast a ray of light on the question of the development of lupus and help in finding better treatments.

How does lupus progress?

In the most typical cases, a young woman will during a few months develop a range of symptoms that may include increasing fatigue, unexplained weight loss, mild fever and severe pains in the joints, where the joints are not swollen or sensitive. Approximately half of the people suffering from lupus will in the early stages also develop a rash on their face, in the form of butterflies, for which people once thought that it resembled a wolf's face (hence the name lupus which is Latin for wolf). The characteristic rash may aid in setting a diagnosis.

It is possible that at a later stage of the disease other parts of the body also become affected, for example the digestive tract (nausea, abdominal pain), blood cells (anemia), the tissue that surrounds the heart and lungs (inflammation) and the nervous system (headache, seizures, strikes and hallucinations). The kidneys are a particularly important target and damage to the organs can lead to kidney failure in later stages of lupus.
Other health problems that may arise in the later stages of lupus include problems such as weakening of the memory and difficulties solving simple math problems. In addition, people suffering from the disease carry the risk of a heart attack with possible fatal consequences, where the heart attack happens as a result of atherosclerosis..

Before corticosteroids became available, there was a high percentage of mortality as a result of lupus. Most people with serious kidney damage would die, and those who were not suffering from kidney disease would survive in only 50 percent of cases.

How is lupus diagnosed?

Since it can affect many different parts of the body, lupus can be similar to many conditions, which makes diagnosis increasingly difficult, especially during the early stages of the disease. The butterfly-shaped rash can certainly help a physician in the diagnosis, but a final diagnosis sometimes depends on two tests that prove the presence of antibodies in the blood. These antibodies, of which one is specific for the core and one for the DNA, attack the core of the patient's cells.

"Sticky blood" may be the culprit

There was a major advance in 1983 in the United Kingdom, with the discovery of "sticky blood", i.e, antiphospholipid syndrome, also known as Hughes syndrome because it was first described by Dr. Graham Hughes, Head of the Lupus Research Unit at St. Thomas' Hospital in London. This syndrome is now recognized as a significant part of the symptoms in some patients who suffer from lupus.
Symptoms vary - they can be headaches, migraines, memory loss and fatigue, as well as blood clots in the veins and more serious problems. Antiphospholipid syndrome has become an important issue for doctors, because they realized that not all manifestations of lupus require steroid treatment. Sometimes, in milder cases, only aspirin is required for blood thinning, or warfarin in cases when blood clots are the major problem. There are many successful cases where the patients' condition improved after they had begun taking warfarin. Dr. Hughes says: "I have always believed that one day it will be accepted that the antiphospholipid syndrome is more common than lupus."

Litmus test for lupus

Almost all patients who suffer from lupus test positive for Anti-Nucleus Antibodies (ANA) and it is therefore very useful to test for them in cases of this disease. However, people suffering from other types of arthritis, including rheumatoid arthritis, may also get a positive result to this test. That is why patients suspected to be suffering from lupus, and who were positive on the test for ANA, are also tested for antibodies to DNA, highly specific for this disease.

What to do?

Lupus can in any form be a very serious disease. The positive thing is that most people who suffer from lupus can be successfully treated. Methods of treating lupus have improved significantly, especially over the past 30 years, and some of the worst complications - like kidney failure - can now be almost always avoided.

Lupus and fertility

Since most people diagnosed with lupus are young women, many of them are worried about whether the disease could reduce their fertility. And here the news is for the most part reassuring: among women who suffer from severe forms of lupus, the disease can indeed reduce fertility and cause more frequent abortions. However, most women who suffer from lupus can get pregnant and give birth to healthy babies.

How is lupus treated?

Milder forms of lupus can usually be kept under control by using non-steroidal anti-inflammatory drugs such as aspirin or ibuprofen, which reduce inflammation and pain. In more severe cases, when lupus attacks the kidneys or other vital organs, patients are usually given steroids - powerful anti-inflammatories that can also cause serious side effects. Approximately two thirds of patients who suffer from lupus are treated with corticosteroids. Patients who suffer from the most serious cases of lupus are treated with immunosuppressives - strong drugs used to prevent the rejection of transplanted organs. They work in a similar way with patients who suffer from lupus, suppressing the immune system to attack the organs of its own body.