Hughes' syndrome (antiphospholipid
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
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
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
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 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.