SYSTEMIC LUPUS ERTHEMASTOSUS?

Firstly, we would like to make it clear that although lupus is a complex disease, and may affect every part of the body, this is most unlikely and most patients suffer from a small number of the following symptoms.

The disease was first described by a French skin specialist in the 19th century as a skin disorder. Half a century later another dermatologist noted that the disease also affected internal organs. “Lupus” is Latin for “wolf” and “erythematosus” is Greek for “reddish”. It is a description of the red rash often found on both cheeks and over the bridge of the nose, which, in Europe, was likened to the classical wolf bite. The rash is also in the shape of a butterfly, and the term “butterfly rash” is now more generally used. However, many patients do not develop the rash.

Discoid lupus is a characteristic rash without any significant problems. It can however sometimes be the forerunner to systemic lupus.

Systemic Lupus Erythematosus (SLE) is an auto-immune disease – one of a number of such diseases such as rheumatoid arthritis, pernicious anaemia, etc., in which the body produces antibodies which damage its own tissues, and can affect many body organs as well as the skin.

The body normally produces antibodies against substances it considers foreign, such as germs, bacteria and viruses and the body may reject grafts or organs from other people – graft rejection – as the immune system recognizes these grafts as foreign.

In SLE, for reasons unknown, the immune system develops a fault; it fails to recognize “self” and produces antibodies against connective tissue – collagen (connective tissue binds the body cells together the way mortar binds the bricks of a building).

These antibodies attack tissue, damage it and inflammation results, together with activation of normal repair processes. The connective tissue in blood vessels is particularly vulnerable and the resulting inflammation may impair the vital blood supply to organs, causing damage. As connective tissue and blood vessels are present in all tissues and organs, symptoms may vary enormously, depending where inflammation is most marked at any particular time

WHAT ARE THE SYMPTOMS?

We advise sufferers and their families NOT to read any literature about lupus which is more than five years old, as diagnosis, treatment and prognosis has changed remarkedly in that time.

During active disease, patients usually feel generally unwell, weak and chronically fatigued, and they may lose appetite, have mouth ulcers and hair loss. Some depression may be evident.

Skin rashes, arthritis, fibrositis, rheumatism and anaemia are common, and at times colour changes may occur in the fingers (Raynaud’s phenomenon.) Kidneys, blood, lungs, heart and brain may be affected, singly or in various combinations from time to time. Many symptoms seem to come and go to different areas of the body at different times.

The disease is chronic, punctuated by flares of activity which may be triggered by sunlight infections and by some drugs, or even some foods. The flares may be followed by periods when the disease is in-active or in remission. Periods of remission can last weeks, months, or even years.

WHO GETS LUPUS?

The disease occurs throughout the world and affects all races. The disease occurs at any age, but is most common in young women (15 to 40 years). It also occurs in men, and in children, even as young as 2 1/2 years old. The prognosis in children is no different than in adults, but diagnosis may be more difficult especially in the very young. More women than men get lupus but for some reason the prognosis in men seems to be worse than for women. Lupus is NOT contagious, and while scientists think there is a genetic pre-disposition, it is yet to be proved inheritable.

SLE is now more common than muscular dystrophy, multiple sclerosis, leukaemia and many other well-known diseases. This is attributable to improved diagnostic tests which are uncovering more cases every year.

SO YOU HAVE LUPUS

Most people when told they have lupus ask “Why me?” After that, you and your lupus must learn to live together. Remember, lupus patients can live normal lives. Patients who face their problems squarely usually do better than those who either tend to ignore it or become excessively anxious.

First, accept and follow your doctor’s advice and at the same time take the common-sense measures – an adequate diet, sufficient rest, avoidance of sunlight and other precipitants, light exercise and a sensible lifestyle.

TREATMENT

As the cause is unknown, treatment is directed at suppressing the damaging antibody level as well as the damaging inflammation. The aim is to maximize the normal repair processes until a natural remission occurs. The results of treatment are gratifying, with sufferers usually being able to lead relatively normal lives.

The drugs used are steroids, cortisone-like drugs which have both a suppressive effect on antibody production and a strong anti-inflammatory action. Anti-malarial drugs and non-steroidal anti-inflammatory medication can be helpful. At times more powerful immuno-suppressants are needed. Drug treatment should always be under close medical supervision and when remission does occur, drug withdrawal must be slow and closely supervised by your doctor.

Some patients with SLE may need continuing treatment for years, others need only intermittent treatment for flares, often going long periods without medication. With todays improved diagnostic tests and medications patients can anticipate a much improved quality of life and a near normal life span.

LUPUS IN PREGNANCY

Once women with lupus were advised not to become pregnant because of the risk of a flare and an increased risk of miscarriage. Now, due to research and new treatments more lupus women can have successful pregnancies. Although it is still considered a high risk most women carry their babies full term. About 20 to 25 per cent of lupus pregnancies end in miscarriage compared to 10 to 15 per cent of normal pregnancies. Counseling and planning before pregnancy is important. A lupus patient should have no signs or symptoms of lupus and be taking no medications for at least 6 months before she becomes pregnant.

Some women experience a flare during or after their pregnancy, and those taking corticosteroids are more likely to develop high blood pressure, diabetes, hyperglycemia or kidney complications, so regular care and good nutrition are essential. About 25 per cent of babies of women with lupus are born prematurely.

A special group of lupus patients are those who suffer from Sjogrens Syndrome and the pregnancy presents a need for extra special medical care. These patients have anti-Ro and anti-La antibodies in their blood and these can cross the placenta and cause mild lupus in the baby. This is not SLE and usually does not require treatment. Another special group have blood abnormalities called the lupus anticoagulant and anticardiolipin antibody (Hughes Syndrome). Again treatment can now greatly help with these pregnancies.

Some drugs used to treat lupus should not be used at all during pregnancy because they may harm the baby or cause a miscarriage. Prednisolone, azathioprine (Imuran) Hydroxychloroquine (Plaquenil) and low dose (baby/junior) aspirin are considered safe. Low dose heparin may be used for anticardiolipin antibodies or lupus anticoagulant.

A woman with lupus who becomes pregnant must work closely with both her obstetrician and her lupus doctor to evaluate her individual needs and circumstances. With care and a good partnership between the patient and her doctors pregnancy can be a safe and rewarding experience.

Anthiphospholipid Syndrome

There are many causes which have been linked to recurrent pregnancy loss. One of the less frequently seen associations is known as the antiphospholipid antibody syndrome.

What are antiphospholipid antibodies?

Under normal circumstances, antibodies are proteins made by your immune system to fight substances recognized as foreign by your body. Some examples of foreign substances are bacteria and viruses. Sometimes the body’s own cells are recognized as foreign. In the antiphospholipid antibody syndrome the body recognizes phospholipids (part of a cell’s membrane) as foreign and produces antibodies against them. Antibodies to phospholipids (antiphospholipid antibodies) can be found in the blood of some people with lupus, but they are also seen in people without any known illness. Lupus anticoagulant (LAC) and anticardiolipin antibody (ACA) are the two known antiphospholipid antibodies that are associated with recurrent pregnancy loss.

What is the antiphospholipid antibody syndrome?

Different physicians may use slightly different definitions to diagnose the antiphospholipid antibody syndrome. In general you need to have a positive blood test for either the lupus anticoagulant or the anticardiolipin antibody, on two separate occasions, at least eight weeks apart. In addition to the blood tests you must also have one the following criteria: A history of thrombosis (clots within the blood vessels), thrombocytopenia (low platelet count) or recurrent pregnancy loss. Several other manifestations may be seen, but not always, in patients with the antiphospholipid antibody syndrome which include skin, heart and nervous system abnormalities.

What is the association between antiphospholipid antibodies and pregnancy loss?

Among women with recurrent pregnancy losses antiphospholipid antibodies have reported to be present in 11%-22%. Lupus anticoagulant (LAC) and/or medium to high anticardiolipin antibodies (ACA) have been associated with first, second, and third trimester pregnancy losses.

Who should be tested for antiphospholipid antibody syndrome?

Women who have had a history of recurrent pregnancy losses should be tested for antiphospholipid antibodies in addition to other routine tests. A history of unexplained poor foetal growth and or the early onset of severe pre-eclampsia (toxaemia, also known as high blood pressure in pregnancy) or an unexplained placental abruption are indications for testing. A history of thrombosis (clots in the blood vessels), stroke, heart attack, thrombocytopenia (low platelet count), presence of other autoimmune disorders such as lupus, an abnormal VDRL, or PTT blood tests would suggest the need for testing.

What is the treatment for the antiphospholipid syndrome in pregnancy?

The drug of choice for treatment is Heparin, which is an injection to prevent blood from clotting. It is used in combination with “baby” (low dose) aspirin. In certain cases Prednisone and baby aspirin are used to treat the antiphospholipid antibody syndrome. All medications have side effects and the choice of therapy should be made after the risks and benefits of the treatments have been discussed between the physician and the patient.

These pregnancies should be monitored closely by ultrasound every month to check on feta growth and by antenatal testing (non-stress tests and biophysical profiles) weekly, beginning at 32 weeks gestation. Although there are a few reports of successful pregnancies without treatment, the majority of researchers have reported a 70%-75% success rate with treatment.

NEONATAL LUPUS

When lupus complicates pregnancy, one of the things that frightens prospective and new parents most is the thought that their child might have the mothers disease. Parents who have heard of a condition (wrongly) called “neonatal lupus” often imagine that terrifying things might happen to their child. Most of the time they need not worry. The facts, as we understand them, are as follows:
- Neonatal lupus is very different from systemic lupus erythematosus;
it does not develop into SLE.
- Neonatal lupus is rare.
- In most cases, neonatal lupus is not serious and does not need
to be treated.
- In most cases, neonatal lupus disappears spontaneously
in a few weeks,leaving no after-effects.
- With a blood test, it is possible to tell which women will
not deliver a child who develops neonatal lupus.
- Many children with neonatal lupus are born to mothers who do not
have SLE.

What is neonatal lupus?

The term is used to describe three major symptoms found in newborns. The commonest symptom is a rash. It can take many forms, but is usually scattered over the body, not necessarily on the face. It shows up a few days or weeks after birth, particularly after sun exposure, and usually disappears after a few more weeks, leaving no scar. The rash looks like many other rashes that babies get. It can be identified definitively only by biopsy, but just doing a blood test and waiting for the rash to disappear is all that is needed.

The second commonest symptom is an abnormal blood count: low platelets, anemia, and other abnormalities. Again, this is seldom serious; these abnormalities usually go away in a few weeks with no treatment.

The rarest abnormality, but a serious one, is a heart rhythm abnormality know as congenital heart block. A normal heartbeat starts in the upper heart (the atria or auricles) and travels smoothly through to the bottom of the heart (the ventricles). In heart block, the atrial beat (about 140 times per minute in a newborn) cannot get through to the ventricles because scar tissue blocks its path. The ventricles then have beat on their own (about 60 times per minute in a newborn).

Since the ventricle beat determines the pulse, the baby has an abnormally slow pulse.

Congenital heart block can often be diagnosed between the 15th and 25th week (4th to 6th month) of pregnancy. If the unborn baby has heart block but appears to be doing well, either nothing is done, or a special form of cortisone is given that will go through the placenta to the baby, but it may not make the heart beat normally again. If the baby is not doing well and is big enough to deliver (30 weeks into pregnancy or later), delivery is often the best way of handling the problem. After birth, many babies with congenital heart block lead normal lives with no treatment, but some need pacemakers, and a small number die from heart disease.

Neonatal lupus is not the same as adult lupus. Babies with neonatal lupus do not develop arthritis, fever, kidney or brain disease.

LUPUS IN CHILDREN

About 20-25% of all lupus occurs in children with girl to boy ratio about 3:1 There is a tendency for lupus to run in families and certain inherited factors increase the risk of lupus in some people. Some infants of mothers with lupus are born with neo-natal lupus associated with the anti-Ro antibody in the mother.

The most common age for diagnosis is from 6-7 years, but can be younger. Lupus in childhood ranges from mild to severe and affects each child differently and can be extremely difficult to diagnose. It is very important for early intervention. Talk to your doctor and if you fail to get action seek another opinion.

Symptoms start in different ways. A child may be very ill or have only mild symptoms, the most common are rash, hair loss, fever, headache, fatigue, joint pain and swelling, stomachache, ulcers in mouth and nose, asthma, and illness due to sun overexposure. Decreased appetite and weight loss is common in children during active phases. There can be major organ involvement in childhood lupus but as time goes on the likelihood decreases that new body systems will become involved. It is important to watch for developing infections.

Prednisolone is the most commonly used medication in higher doses than in adults because children metabolize the drug much more quickly. Unfortunately side effects can occur. Many patients become cushingoid, develop acne and excessive facial and body hair. This is particularly distressing for adolescents as their appearance is very important, so it is vital to discuss this as openly as possible with teenagers as these issues are a major source of stress and worry.

Lupus affects the whole family and the goal of therapy is to maintain a normal life. Families that cope best are those who do not place their child in a sick role, and do not limit the child’s activities in daily life. There has been a remarkable improvement in the prognosis of children with lupus due to improvement in diagnosis and treatment. Most importantly, children and their families need to seek careful and competent medical treatment and the support of their school and community.
Awareness is important.

LUPUS IN MEN

Lupus is often called a “woman’s disease” because it occurs 9 times more frequently among adult females than among adult males. However, lupus can occur in either sex, and at any age. It does not differ in any major way in males from the pattern in females. Under the age of 12 and after 60 the ratio is something like three to one. These differing ratios apply only to systemic lupus and not discoid lupus, which affects more men than does SLE. More men seem to develop drug-induced lupus.

Some males have a family history of lupus. The symptoms of SLE are identical in men and women at the time of initial presentation. The treatment for males is the same as for females, but some studies suggest the prognosis is not as good.

There is no significant increase in estrogen in men with lupus. Men with lupus are fertile, sexually active and potent, and have normal reproductive histories. None of these characteristics would be apparent if males with SLE were any different hormonally than males who do not have lupus.

It is possible it may be more difficult for men to cope with having lupus because of expectations put upon them. There may be a decrease in job performance, or they may not even be able to continue