What is Lupus

By Dr. M. Walravens, rheumatologist, O.L. Vrouw-Clinic Aalst

Systemic Lupus
Systemic Lupus defined
Who is affected by lupus?
Prevalence of lupus
Cause and heredity
Onset and symptoms
Diagnosis
Treatment
Drug-induced lupus
Lupus and pregnancy
Neonatal lupus
Important antibodies in lupus
Complaints and symptoms that require medical attention
Evolution and prognosis

Systemic lupus
Also called: "lupus erythematosus desseminatus" (LED) or "systemic lupus erythematosus". Systemic lupus is different from discoid lupus. Discoid lupus is mostly limited to the skin. It can occur by itself or in combination with systemic lupus.
What is systemic lupus?
Systemic lupus is a generalised autoimmune disease. In an autoimmune disease, the immune system (=defence) partly targets itself, by producing autoantibodies that cause inflammatory diseases. Generalised means that the autoimmunity can attack different organ systems and can cause generalised disease manifestations
Who is affected by lupus?
Basically anyone at any age, but mostly lupus strikes women of childbearing age (80 to 90%). The number of lupus patients affected by lupus also differs according to race. In the USA lupus is more common in Blacks than in Caucasians. Genetic (hereditary) factors are sure enough believed to play a role in the development of the disease.
Prevalence of lupus
Studies are scarce and results vary widely, but it cannot be denied that the number of lupus patients is increasing. Improved diagnostic measures and a better understanding of the disease are two important factors in the increase of lupus patients, but there is also an absolute rise in the incidence (= number of new patients per year of a specific disease)
Cause and heredity
The cause remains unknown. Experts are getting convinced of the fact that inflammations and damage are caused by a reaction of autoantibodies with normal cellular components and other elements. In this process both genetic as environmental factors can play a role. Several genetic factors are of importance, but they are unlikely to be entirely passed on. This is the reason why lupus is not a hereditary disease, although risks of developing lupus are bigger if a relative in the first degree has been diagnosed with the disease. Ultraviolet rays are an example of an environmental factor known to trigger lupus. Although it has not been proved, viruses may also play a role in the onset of lupus. Lupus, however, is not contagious at all. Hormones are involved as well: female hormones stimulate lupus, while male hormones protect against it. This is one of the reasons to avoid the use of the contraceptive pill.
Onset and symptoms
Possible symptoms and manifestations of lupus are very diverse. The onset of the disease is never the same: it may start off with any of the symptoms mentioned below, in a mild or severe form. Because there are so many symptoms, we have to stick to the following summary: 90 % fatigue, arthritis and joint pain 80 % fever 70 % hair loss, anaemia, swollen glands 60 % weight loss, poor appetite, butterfly-shaped rash 50 % inflammation of the pleura (pleuritis), inflammation of the heart sac (pericarditis), inflammation of the membrane lining the abdominal cavity (peritonitis), renal involvement, personality changes, purpura 40 % photosensitivity, bacterial infections 30 % ulceration of the mucous membranes e.g. aphthous ulcers, pain in the muscles or myositis (inflammation of the muscles), gastrointestinal complaints, enlargement of the liver, high blood pressure, pneumonia, myocarditis (inflammation of the heart muscle) and endocarditis (involvement of the heart valves). 20 % Raynaud's phenomenon (turning white of the fingers), discoid lupus (skin involvement in lupus with round disc-shaped scars), inflammation of the eyes, Sjogren's Syndrome, severe renal involvement, attacks of epilepsy, psychoses, inflammation of the coronary arteries (supply of blood to the heart muscle) 10 % hives, oedema or formation of blisters on the skin, lupus pneumonia, brain damage or damage to the spinal cord, migraine, autoimmune destruction of red blood cells, low platelet count, neuritis (inflammation of the nerves)
Diagnosis
The diagnosis is based on complaints and/or symptoms and/or involvement of internal organs and/or abnormalities in laboratory test results. There is no single lupus test that can confirm the disease. Beginning mild forms of lupus are hard to diagnose, and sometimes diagnosis is only possible after following the evolution of the symptoms. In case of a possible onset of lupus, following up the early symptoms as well as informing the patient are very important. Because there is no decisive test for lupus and due to the changing character of the disease, criteria have been defined to classify lupus. Their main purpose, however, is scientific research as they can only be used as a guideline to diagnose lupus. In some cases lupus can be diagnosed before the patient meets these criteria.
Treatment
Must be focused on the following: general degree of illness and complaints internal organs involved degree of involvement of these organs Medications range from absolutely nothing to high doses of corticosteroids or immunosuppressive drugs. Antimalarials and nonsteroidal anti-inflammatory drugs are frequently used. The basic principle to treat lupus is to react quickly with high doses of corticosteroids and/or immunosuppressive drugs if necessary and maintain the lowest dose possible during periods of low disease activity.
Drug-induced lupus
Some 50 prescription drugs can produce symptoms of lupus, which normally disappear after the patient stops taking the medication. This does not imply, however, that these drugs are forbidden for lupus patients. The most important ones are: Hydralasine (Neprosol), Procaïnamide (Pronestyl), Isoniazide (Rimifon), Propylthiouracil (Strumazol), d-Penicillamine (Kelatin) The prescription drugs mentioned may have other brand names
Lupus en zwangerschap
A lupus patient is allowed to become pregnant if the disease is under control, with a low dose of medication if necessary. The safest drug during pregnancy is a low dose of corticosteroids. A lupus patient should not become pregnant without a complete check-up, given the risk for mother and child. The child may have neonatal lupus due to the presence of anti-Ro antibodies and for the mother there is the risk of a severe flare. During pregnancy a flare may occur. In this case, it needs to be treated appropriately and the best way to do that is with corticosteroids. If you have lupus, there is a greater risk of miscarriage, often due to anti-cardiolipin antibodies (read further). These antibodies cause the blood to clot more easily, so that small blood vessels in the placenta can get clogged up. Low weight at birth occurs more often in newborn babies of lupus patients.
Neonatal lupus
This is a kind of skin lupus that breaks out a few weeks after birth and disappears at the age of 6 months. It is caused by the transfer of anti-Ro antibodies through the placenta to the foetus. In more severe cases, the heart of the foetus may be affected at about 18 weeks and the foetus may die, or the baby may be born with a congenital heart block (delayed contraction of the ventricle due to a conduction disorder, resulting in a very slow heart rate). The survival rate of these babies varies. The children themselves do not have lupus.
Most important antibodies in lupus
Anti-Sm antibodies
About 15 % of lupus patients in Europe have anti-Sm, compared to 30 % in the USA. It is a disease marker: this means it is only found in patients with lupus, and mostly in relatively serious cases.
Anti-dsDNA antibodies
Seen in about 40 % of all lupus patients. In more severe cases of lupus the percentage amounts to 80 % during a flare. These antibodies are tightly linked with lupus and lupus nephritis, though this is not 100 % the case. They occur especially in lupus nephritis, in which they are also believed to play an important role.
Anti-Ro
About 40 % of people with lupus have anti-Ro. These antibodies are linked with congenital heart block and neonatal lupus and are probably the cause of it. (read pregnancy)
Anticardiolipin antibodies
Often facilitate blood clotting, which may lead to a propensity for miscarriage and thromboses in young patients.
Antibodies against red blood cells (positive Coombtest)
kMay cause haemolysis, or the destruction of red blood cells on an autoimmune basis. Severe autoimmune haemolysis is very rare.
A whole range of other antibodies
... of which the importance is not yet clear.
Signs and symptoms that need immediate medical examination:
fever attacks sudden onset of extreme fatigue general malaise neurological complaints extreme headache or unexplainable mood swings acute or subacute skin rashes unexplainable chest pains unexplainable abdominal pains new complaints due to arthritis increasing shortness of breath presence of protein in the urine any form of infection
Evolution and prognosis
Systemic lupus progresses with ups and downs. Periods of serious exacerbations may alternate with periods of low disease activity, in which one may even discontinue taking medication. The patient is then said to be in remission. After menopause, lupus is likely to disappear spontaneously. Life expectancy has increased greatly over the last 40 years and statistically almost equals a normal life span with 93 % of all patients surviving ten years after diagnosis. This improvement is due to early diagnosis and better use of medication. Even lupus nephritis can be reversed if treatment follows immediately and intensively. The more severe forms of lupus that lead to death by affecting the brain or kidneys have become rare. Exceptionally, however, complications (mostly infections) or a neglected flare may be fatal. Once "systemic lupus" is diagnosed, adapted drug treatment should be able to stabilise the disease. Still, lupus is to be considered a serious disorder. Accurate compliance of medical advice together with regular medical check ups are absolutely necessary to control the disease. Clear information is in this case essential. Also contact with people from our support group, the CIB-league, can be a step forward in the process of learning how to cope with lupus.

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