SYSTEMIC LUPUS ERYTHEMATOSUS
Lupus also known as
SLE, can affect all parts of the body through
inflammation of the connective tissues, also known as
collagen.
The immune system normally
makes antibodies to protect against viruses and bacteria.
In an autoimmune disorder such as lupus,
the immune system loses its ability to tell the
difference between bacteria and its own cells and
tissues.
The immune system then makes antibodies directed against
itself.
These so-called auto-antibodies react with 'self
antigens' to form immune complexes, which build up in the
tissues and cause inflammation, injury to tissues, and
pain.
Lupus can affect all or any part of the body, including
the joints, skin, kidneys, heart, lungs, blood vessels,
and brain.
Although people with the disease may have many different
symptoms, some of the most common ones include:
Arthritis in
lupus commonly affects the knees, wrists and fingers with
Osteoporosis being a frequent complication of the disease
and its therapy.
ie: Spinal osteoporosis may be attributable to
steroids.
Lupus is also known as a
rheumatic illness.
The rheumatic diseases are
a group of disorders that cause aches, pain, and
stiffness in the joints, muscles, and bones At present,
there is no cure for lupus.
However, the symptoms of lupus can be controlled
with appropriate treatment, and most people with the
disease can and do lead active, healthy lifestyles with
normal life spans.
Intense research is
underway and scientists are continuing to make great
strides in understanding lupus which may ultimately, lead
to a cure.
BACK
RENAL LUPUS
Urinary, or functional
renal abnormality directly related to the activity of
lupus, occurs in about half of SLE patients.
Almost all lupus patients
have some degree of renal involvement related to their
condition, but a considerably smaller proportion of these
patients actually progress to end-stage renal disease .
Lupus patients, like the
rest of the population, are also susceptible to other
primary renal insults that may significantly contribute
to the deterioration in their renal function.
Inflammation of the
kidneys (lupus nephritis) can impair
their ability to effectively get rid of waste products
and other toxins from the body.
This inflammation can be infectious or noninfectious and
is treated accordingly by the primary care physician.
There is usually no pain associated with kidney
involvement, although some patients may notice fluid
retention peripherally and around the facial areas.
Most often the only indications of kidney disease is
abnormal urine tests (protein, creatnine and urea
results), high blood pressure and possibly high
cholesterol as well as fatigue.
Urinary abnormalities occurring are regularly monitored
and treated when necessary.
BACK
CNS LUPUS
CNS involvement is
significantly associated with the antiphospholipid
syndrome (APS).
Common symptoms of CNS lupus are:
Headaches
Memory
disturbances
Dizziness
Vision problems
Some of these symptoms,
however, also can be caused by some treatments of lupus
or by the emotional stress of dealing with the disease.
Headaches are frequent, and their organic cause is
suggested by their onset which may include sudden
development and associated double vision, a migraine
intensity and possibly a personality change.
Peripheral neuropathy occurs in about 10% of patients,
and can involve the cranial nerves and multiple
individual nerves.
Neuropsychiatric Lupus, the more
acceptable medical term for cns lupus symptoms, can also
include depression, anxiety and psychotic behaviour as
symptomatic of the illness.
BACK
SUBACUTE CUTANEOUS LUPUS
This clinically distinct form of
cutaneous LE has been analyzed as a separate
entity and thus, its clinical importance has been
more appreciated as a subset of SLE.
These patients frequently have a
mild systemic illness marked by musculoskeletal
complaints and serologic abnormalities.
Patients have in common a
recurring, superficial, nonscarring type of
cutaneous lupus erythematosus (LE) that occurrs
in a characteristic distribution.
SCLE is usually diagnosed through
a combination of signs and/or symptoms.
As with SLE, there really is no
one symptom/sign that is absolutely diagnostic.
The bloodwork, the way the rash
looks, the character of the rash, the pathology
of the rash, systemic symptoms, etc. are all
taken into consideration.
As SCLE rash can be itchy
or not, scarring in some instances,
psoriasis-like it is most usually affected by UV
radiation and a report on an SCLE rash would
reflect inflammation.
It is a rash seen in about 9% of
lupus patients; 20% with SCLE also have lesions
typical of discoid lupus. SCLE doesn't scar the
skin, & the lesions may itch.
Cutaneous vasculitis is a common
occurrance with SCLE.
SCLE is treated using
antimalarials, steroids, cytotoxic drugs,
sunprotection, & cortisone creams.
BACK
ANA NEGATIVE LUPUS
A high incidence
of both photosensitive and cutaneous involvement.
Arthritis and
Raynaud's phenomena are common.
There is a low
frequency of both renal and CNS involvement.
Many patients have
a positive rheumatoid factor.
Prognosis is
better than that given to patients who are
diagnosed with positive ANAs.
BACK
DRUG INDUCED LUPUS
A drug-induced lupus-like
syndrome is characterized
by mild manifestation of the following symptoms:
DISCOID LUPUS ERYTHEMATOSUS
Discoid lupus
erythematosus which primarily affects
the skin is manifested
as a red, sometimes raised rash, which may become thick
and scaly, and can appear on the face, scalp or torso.
The rash may last for days or years and may undergo
remissions with recurrence.
A small percentage of discoid lupus patients develope
systemic involvement or SLE.
Alopecia is common with discoid lupus.
When photosensitivity is involved, a dermatologist
usually provides aggressive treatment in order to prevent
permanent scarring and extensive lesions.
Many patients with discoid lupus have no other systemic
involvement and the prognosis leaves the patients living
good quality lives over normal life spans.
BACK
PHOTOSENSITIVITY
Sun screens are effective
against UVA and UVB although complete avoidance of the
sun is the best course.
When a patient has discoid involvment caused by the sun,
topical steroids are one of the common therapies used.
Antimalarials which have sunblocking anti-inflammatory
and immunosuppressive effects are the usual prescribed
treatment.
If more vigorous treatment is required, low dosage
steroids or cytotoxic drugs are tried.
BACK
SJOGREN'S SYNDROME
Extreme dryness of mucosal
areas and usually determined through a positive
Schirmers/litmus test with an opthamologist during a
regular eye exam which with Sjogrens Syndrome should be
every 6-9 months..
In Sjogren's syndrome, the
body's immune system mistakes its own moisture producing
glands for foreign invaders.
The immune system then
attacks and destroys these glands causing the typical
symptoms of dry eyes and dry mouth.
This syndrome can mimic other diseases as well as
occuring in a cross-over diagnosis as secondary to
another primary autoimmune illness such as lupus, thereby
making diagnosis difficult much like with lupus.
In addition to affecting the eyes and mouth, it can cause
skin, nose and vaginal dryness and can affect the other
organs of the body, including kidneys, blood vessels,
lungs, liver, pancreas and brain.
There are many treatments available to treat Sjogren's
Syndrome including corticosteroids and artificial tear
and saliva products, both oral and systemic.
Also there are punctal plugs for dry eyes which can be
inserted in the eye specialist's office and are
successful by retaining any fluid you put into your eyes
such as artificial tears. There are 4 punctum ducts, 2
upper and 2 lower which can be plugged. It can be a
temporary or permanent treatment depending on the results
as determined by the patient.
Like lupus, stress is a triggering factor for activity or
flaring of the illness.
Photosensitivity can be a problem as it is in lupus, and
the sun should be avoided as much as possible within
reason.
The majority of sjogren patients are again, as in lupus,
women.
Cause and cure of the disease is still unknown, to date.
BACK
RAYNAUD'S PHENOMENA
Raynaud&rsquos phenomenon is
a condition that results in the discoloration of
the fingers and/or the toes when the patient is
exposed to changes in temperature (cold or hot)
or emotional events.
Skin discoloration occurs because
an abnormal spasm of the blood vessels results in
a diminished blood supply.
Initially, the fingers involved
turn white because of diminished blood supply.
The fingers then turn blue
because of prolonged lack of oxygen.
Finally, the blood vessels
reopen, causing a local flushing phenomenon,
which turns the fingers red.
This three-phase color sequence
(white to blue to red), which occurs most often
upon exposure to cold temperature, is
characteristic of Raynaud&rsquos phenomenon.
Persons with Raynaud&rsquos
phenomenon should minimize their exposure to
extremes of temperature (particularly cold) and
rapid changes of temperature.
BACK
PREGNANCY WITH LUPUS
Currently, 50% of all
lupus pregnancies are completely normal, 25% deliver
normal babies prematurely, and spontaneous abortion
(miscarriage) or death of the baby accounts for the
rerighting 25%.
Pregnancy is advised against during flares
as well as when major organ involvment is present.
Women conceiving after
5 or 6 months of remission are much less likely
to experience lupus flares during her pregnancy than are
those women who get pregnant while their lupus is active.
Lupus nephritis before conception also increases the
chance of experiencing a lupus flare during pregnancy.
Women in remission have
much less trouble than do women with active disease and
their babies do much better also.
Aspirin is safe and it is most often used to protect
against a complication known as toxemia of pregnancy.
Cyclophosphamide (Cytoxan) is definitely harmful if taken
during the first three months of pregnancy.
Flares most often occur
during the first or second trimester, or during the two
months immediately after delivery.
The most common symptoms of flares during pregnancy are
arthritis, rashes and fatigue.
Antibodies that interfere with the function of the
placenta are called antiphospholidid antibodies.
These antibodies can occur during the second trimester,
slowing the baby's growth.
Aspirin, Prednisone, Heparin, and Plasmapheresis have all
been suggested as possible therapies for the
antiphospholipid antibodies.
It is important to
distinguish the symptoms of a lupus flare from the normal
body changes that occur during pregnancy.
Frequent doctor visits are
important in any high risk pregnancy because many
conditions which may occur can be prevented or treated
more easily when found early.
Prednisone does not get
through the placenta and is safe for the baby.
Specifically,
dexamethasone and betamethasone do reach the baby and are
used only when it is necessary to treat the baby as well.
A few doctors feel that
all pregnant women with lupus should take small doses of
Prednisone to prevent early abortion.
Similarly, some physicians feel steroids should be given
or increased after the baby is born to prevent
post-partum flare.
It is important to
note that although many lupus pregnancies will be
completely normal,
all lupus pregnancies should be considered
high-risk.
High-risk is a term commonly used by obstetricians to
indicate that solvable problems may occur and should be
anticipated.
Pregnant lupus patients should be managed by
obstetricians who are thoroughly familiar with high risk
pregnancies and work in close concert with the woman's
other physicians.
Patients with Lupus and Sjogren&rsquos
syndrome who plan to become pregnant should consult with
their doctor about being tested for specific
auto-antibodies (ANAs) that may be present in their
blood.
These antibodies, anti-Ro (SS-A) and anti-La (SS-B), in
rare cases, have been associated with heart problems in
newborns, termed neonatal lupus syndrome.
When NEONATAL LUPUS Occurs:
Neonatal lupus is not SLE in babies.
Neonatal lupus is the only type of congenital abnormality
found in children of mothers who have lupus and it can
also cause a rash on the infant which clears up within a
few months.
About 3% of all pregnant lupus women
will have a baby with a syndrome known as neonatal lupus.
Neonatal lupus consists of a transient rash, transient
blood count abnormalities, and a special type of heart
beat abnormality which is very rare and which is called
heart block.
If the heart beat abnormality occurs, it is treatable
although it is permanent.
For babies with neonatal lupus who do not have the heart
problem, there is no trace of the disease by 6 months of
age and it does not recur nor does it predispose the
child to lupus later in life.
Even babies with the heart beat abnormality problem
(heart block), have normal growth and development, living
normal lives.
BACK
VASCULITIS
Vasculitis is an inflammation of the
blood vessels.
Inflammation is a condition in which tissue is damaged by
blood cells entering the tissues.
These are mostly white blood cells which circulate and
serve as our major defense against infection.
Ordinarily, white blood cells destroy bacteria and
viruses.
However, they can also damage normal tissue if they
invade it.
Vasculitis can affect very small blood vessels
(capillaries), medium-size blood vessels (arterioles or
venules), or large blood vessels (arteries and veins).
Several things can happen to an inflamed blood vessel.
If it is a small vessel, it may bread and produce tiny
areas of bleeding in the tissue.
These areas will appear as small red or purple dots on
the skin.
If a larger vessel is inflamed, it may swell and produce
a nodule which may be felt if the blood vessel is close
to the skin surface.
The inside of the vessel tube may become narrowed so that
blood flow is reduced, or the inside may become totally
closed (usually by a blood clot which forms at the site
of inflammation).
If blood flow is reduced or stopped, the tissues which
receive blood from that vessel begin to die
Substances which cause allergic relations are called
"antigens."
They cause the body to make proteins called
"antibodies" which bind to the antigen for the
purpose of getting rid of it.
Antigen and antibody bound together are called
"immune complexes."
Two primary ways in which immune complexes destroy
antigens are
by attracting white blood cells to digest the antigen,
and
by activating other body substances to help destroy the
antigens.
Unfortunately, some immune complexes do not serve their
purpose of destroying antigens.
Instead, they reright too long in the body and circulate
in the blood and deposit in tissues.
They commonly accumulate in blood vessel walls, where
they cause inflammation.
It is likely that some white blood cells which kill
infectious agents (cytotoxic cells) can also accidentally
damage blood vessels and cause vasculitis.
In the vasculitis caused by lupus, the antigens causing
the immune complexes are often not known.
In some cases, the complexes contain DNA and anti-DNA
antigens, or Ro (also called SS-A) and anti-Ro antigens.
A recently discovered antibody, ANCA (anti-neutrophil
cytoplasm antibody), can cause vasculitis in some
individuals.
The diagnosis of vasculitis is based on a person's
medical history, current symptoms, a complete physical
examination, and the results of specialized laboratory
tests.
Blood abnormalities which often occur when vasculitis is
present include an elevated sedimentation rate, anemia, a
high white blood count and a high platelet count.
Blood tests can also be used to identify immune complexes
or antibodies that cause vasculitis in the circulation
and measure whether complement levels are abnormal.
These tests take several days to complete.
The physician may also order a urine analysis.
If there are any symptoms that suggest heart involvement,
tests that may be ordered include: EKG, ECHO cardiogram
and heart scans.
For lung symptoms, the physician may order a chest x-ray,
obtain blood from an artery to measure the oxygen content
and blood gases, and schedule a pulmonary function test.
A pulmonary function test uses a specialized machine to
measure how well the lungs handle air and oxygen as you
breathe into it.
If there are abdominal symptoms, the physician may order
ultrasound or CAT scans of the organs in the abdomen, or
other special x-rays to see the intestines.
For brain symptoms, CAT scans and magnetic resonance
images are frequently useful.
Many cases of vasculitis do not require treatment.
For example, a few spots on the skin now and then (if not
combined with other symptoms) may not require any
medications.
Most physicians recommend cortisone-type medications,
such as Prednisone, Prednisolone, or methylprednisolone
(Medrol) as the initial treatment for vasculitis.
Some people with severe vasculitis or vasculitis that
does not respond well to cortisone-type drugs will need
to be treated with cytotoxic drugs.
These medications kill the cells that cause inflammation
in the blood vessels.
For the vast majority of people with vasculitis,
treatment is very effective and prognosis is good.
The vasculitis may disappear only to reoccur later and
require treatment again; or it may be suppressed but
never really go away, so that some ongoing treatment is
always required as with lupus.
BACK
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