What is 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, www.LupusMCTD.com can be a step forward in the
process of learning how to cope with lupus.
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