- 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 bleed and show as
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.
For example, a person with vasculitis of a
medium-sized artery in the hand may develop a
cold finger which hurts whenever it is used and
occasionally this can progress to gangrene.
- Vasculitis can be caused by
an auto-immune reaction.
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.
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.
- Vasculitis can occur
in many different illnesses.
Some of the illnesses that can cause vasculitis
are:
Infections
Lupus
Rheumatoid Arthritis
Polymyalgia Rheumatica
Scleroderma
Wegener's Granulomatosis
Temporal Arteritis
Cryoglobulinemia
Erythema Nodosum
Tumors
Leukemia
Lymphoma
Vasculitis can also occur of unknown etiology,
without any other obvious associated infection or
other illnesses related.
- Vasculitis can cause many
different symptoms, depending upon what tissues
are involved and the severity of the tissue
damage.
Some patients are not ill and notice only
occasional spots on their skin.
Others are very ill with systemic symptoms and
major organ damage.
A list of symptoms based on the tissues in which
vasculitis occurs
include the systemic symptoms of
fever, generally feeling bad
("malaise"), muscle and joint pain,
poor appetite, weight loss, and fatigue.
This set of complaints can occur in many
illnesses and is not specific to vasculitis.
On the skin can be seen red or purple dots
(petechiae),
usually most numerous on the legs.
When the spots are larger, about the size of the
end of a finger, they are called purpura.
Some look like large bruises.
These are the most common vasculitis skin
lesions, but hives, itchy lumpy rash, and painful
or tender lumps can occur.
Areas of dead skin can appear as ulcers
(especially around the ankles), small black spots
at the ends of the fingers or around the
fingernails and toes (nail fold infarcts), or
gangrene of fingers or toes.
Aching in joints and a frank arthritis with pain,
swelling and heat in joins. Deformities resulting
from this arthritis are rare.
In the brain, vasculitis can cause many problems,
from mild to severe.
They include headaches, behavioral disturbances,
confusion, seizures, and strokes.
See: (PsychoNeurological/CNS
Lupus)
Peripheral nerve symptoms may include numbness
and tingling, usually in an arm or a leg, or in
areas which would be covered by gloves or socks,
and possible loss of sensation or loss of
strength, especially in the feet or hands.
Inadequate blood flow in the intestines can cause
crampy abdominal pain and bloating.
If areas in the wall of the intestine develop
gangrene, blood will appear in the stool.
If the intestinal wall develops a hole (called a
perforation), surgery may be required.
Vasculitis in the coronary arteries is unusual in
lupus.
If it occurs, it can cause a feeling of heaviness
or aching in the chest during exertion (angina),
which is relieved by rest.
Heart attacks rarely occur as a result of
vasculitis.
Vasculitis in the lung tissue can cause
pneumonia-like attacks (pneumonitis) with chest
x-ray changes that look like pneumonia, and
symptoms of fever and cough.
Occasionally, inflammation can lead to scarring
of lung tissue with chronic shortness of breath
and may result in COPD.
Vasculitis is not common in kidneys of people
with lupus, even those who have lupus nephritis.
It may not cause any symptoms, although most
patients with renal vasculitis have high blood
pressure.
Vasculitis involving the small blood vessels of
the retina can occur in lupus.
The retina is a tissue at the back of the eye
which contains cells that have to be activated to
form a visual image.
Sometimes, vasculitis of the eyes causes no
symptoms.
Usually however, there is visual blurring which
comes on suddenly and stays, or a person may even
lose a portion of their vision.
In other non-lupus types of vasculitis, such as
temporal arteritis, there is sudden loss of part
or all of the vision in one eye, usually
accompanied by a severe headache.
- If you suspect that you or
a friend or relative has vasculitis, you should
consult a physician as soon as possible as
vasculitis can be very mild and of little
importance, or very severe and life-threatening,
and any degree in between.
Therefore, an expert should help you decide:
(a) if you have vasculitis
(b) how serious it is
(c) if and/or how it should be treated
Doctors trained in several different specialties
are taught to recognize and treat vasculitis.
These include the following Specialists:
rheumatologists, general internists,
dermatologists, hematologists, nephrologists,
gastroenterologists, infectious disease experts,
pulmonologists, cardiologists, geriatricians,
neurologists, and ophthalmologists.
- 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 (and urine analysis) can also be
used; both to identify immune complexes or
antibodies that cause vasculitis in the blood
circulation and measure whether complement levels
are abnormal.
These tests take several days to complete.
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
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.
Sometimes, inflammation in medium and large-size
arteries or veins can be seen by injecting dye
into them and viewing the outlines of the blood
vessels on x-ray.
This procedure is called an
"angiography."
It can be done in any area of the body.
The diagnosis is most firmly made by seeing
vasculitis in involved tissue.
Your physician may suggest taking a biopsy of the
involved tissue and examining that tissue under a
microscope.
Finally, it may be important for your physician
to consult with other medical specialists about
your case.
For example, if your physician is a
rheumatologist and you have visual complaints
which could be indicative of vasculitis, you may
be referred to an ophthalmologist.
It is very important that one physician be in
charge of your case, coordinating your care and
helping you with decisions.
- The choice of treatment
for vasculitis depends on the severity of the
vasculitis, your general health, and your past
reactions (positive and negative) to medications.
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 will to cortisone-type
drugs will need to be treated with cytotoxic
drugs.
These medications kill the cells that cause
inflammation in the blood vessels.
The two most frequently used are azathioprine
(Imuran) and cyclophosphamide (Cytoxan).
They are usually used in combination with
Prednisone and are often effective in treating
vasculitis.
Procedures
that have been helpful in treating some cases of
vasculitis include: plasmapheresis, intravenous
gammaglobulin, and cyclosporin (a medication used
to prevent organ rejection in transplant
patients) and stem-cell transplant.
Your physician can provide you with current
information.
- There are various
outcomes for people suffering from vasculitis.
For many patients, vasculitis, especially if
confined to the skin, may be annoying but never
life-threatening.
For those individuals, life can be normal.
On the other hand, a small number of people have
severe vasculitis involving major organ systems.
In these cases, damage can occur so rapidly that
treatment does not have time to work or the
condition may be resistant to treatment.
An attack of vasculitis can be fatal or
permanently disabling for individuals so
affected.
For the vast majority of people with vasculitis,
treatment is very effective.
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 may always be required.
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