Types of Vasculitis
There are many types of vasculitis. Each type
involves inflamed blood vessels. However, most types differ in whom they affect
and the organs that are involved.
The types of vasculitis often are grouped based on
the size of the blood vessels they affect.
Mostly Large Vessel Vasculitis
These types of vasculitis usually, but not always,
affect the larger blood vessels.
Behçet's Disease
Behçet's (beh-CHET'S) disease can cause recurrent,
painful ulcers in the mouth, ulcers on the genitals, acne-like skin lesions,
and eye inflammation called uveitis (u-ve-I-tis).
The disease occurs most often in people aged 20 to
40. Men are more likely to get it, but it also can affect women. It's more
common in people of Mediterranean, Middle Eastern, and Far Eastern descent,
although it rarely affects Blacks.
Researchers believe that a gene called the HLA-B51
gene may play a role in Behçet's disease. However, not everyone who has
the gene gets the disease.
Cogan's Syndrome
Cogan's syndrome can occur in people who have a
systemic vasculitis that affects the large vessels, especially the aorta and
aortic valve. The aorta is the main artery that carries oxygen-rich blood from
the heart to the body. A systemic vasculitis is a type of vasculitis that
affects you in a general or overall way.
Cogan's syndrome can lead to eye inflammation called
interstitial keratitis (in-ter-STISH-al ker-ah-TI-tis). It also can cause
hearing changes, including sudden deafness.
Giant Cell Arteritis
Giant cell arteritis (ar-ter-I-tis) usually affects the
temporal artery, an artery on the side of your head. This condition also is
called temporal arteritis. Symptoms of this condition can include headache,
scalp tenderness, jaw pain, blurred vision, double vision, and acute (sudden)
vision loss.
Giant cell arteritis is the most common form of
vasculitis in adults older than 50. It's more likely to occur in people of
Scandinavian origin, but it can affect people of any race.
Polymyalgia Rheumatica
Polymyalgia rheumatica (POL-e-my-AL-ja RU-ma-ti-kuh), or PMR,
commonly affects the large joints in the body, such as the shoulders and hips.
PMR typically causes stiffness and pain in the muscles of the neck, shoulders,
lower back, hips, and thighs.
Most often, PMR occurs by itself, but 1020
percent of people who have PMR also develop giant cell arteritis. Also, about
half of the people who have giant cell arteritis also can have PMR.
Takayasu's Arteritis
Takayasu's (tock-e-AH-sues) arteritis affects
medium- and large-sized arteries, particularly the aorta and its branches. The
condition is sometimes called
aortic arch syndrome. Though rare, Takayasu's arteritis
occurs mostly in teenage girls and young women. The condition is more common in
Asians, but it can affect people of all races and occur throughout the world.
Takayasu's arteritis is a systemic disease. A
systemic disease is one that affects you in a general or overall way. Symptoms
of Takayasu's arteritis may include tiredness and a sense of feeling unwell,
fever, night sweats, sore joints, loss of appetite, and weight loss. These
symptoms usually occur before other signs develop that point to arteritis.
Mostly Medium Vessel Vasculitis
These types of vasculitis usually, but not always,
affect the medium-sized blood vessels.
Buerger's Disease
Buerger's disease, also known as thromboangiitis obliterans,
typically affects blood flow to the hands and feet. In this disease, the blood
vessels in the hands and feet tighten or become blocked. This causes less blood
to flow to the affected tissues, which can lead to pain and tissue damage.
Rarely, Buerger's disease also can affect blood
vessels in the brain, abdomen, and heart. The disease usually affects men aged
20 to 40 of Asian or Eastern European descent. The disease is strongly linked
to cigarette smoking.
Symptoms of Buerger's disease include pain in the
calves or feet when walking, or pain in the forearms and hands with activity.
Other symptoms include blood clots in the surface veins of the limbs and
Raynaud's
phenomenon.
In severe cases, ulcers may develop on the fingers
and toes, leading to gangrene. The term "gangrene" refers to the death or decay
of body tissues.
Surgical bypass of the blood vessels may help
restore blood flow to some areas. Medicines generally aren't effective
treatments. The best treatment is to stop using tobacco of any kind.
Central Nervous System Vasculitis
Central nervous system (CNS) vasculitis usually
occurs as a result of a systemic vasculitis. A systemic vasculitis is one that
affects you in a general or overall way.
Very rarely, vasculitis affects only the brain
and/or spinal cord. When it does, the condition is called isolated vasculitis
of the CNS or primary angiitis of the CNS.
Symptoms of CNS vasculitis are headache, problems
thinking clearly or changes in mental function, or stroke-like symptoms, such
as muscle weakness and paralysis (an inability to move).
Kawasaki Disease
Kawasaki
(KAH-wah-SAH-ke) disease is a rare childhood disease in which the walls of
the blood vessels throughout the body become inflamed. The disease can affect
any blood vessel in the body, including arteries, veins, and capillaries.
Kawasaki disease also is known as mucocutaneous lymph node syndrome.
Sometimes the disease affects the coronary arteries,
which carry oxygen-rich blood to the heart. As a result, a small number of
children who have Kawasaki disease may develop serious heart problems.
Polyarteritis Nodosa
Polyarteritis nodosa (POL-e-ar-ter-I-tis no-DO-suh) can affect
many parts of the body. It often affects the kidneys, the digestive tract, the
nerves, and the skin.
Symptoms often include fever, a general feeling of
being unwell, weight loss, and muscle and joint aches, including pain in the
calf muscles that develops over weeks or months. Other signs and symptoms
include
anemia
(a low red blood cell count), a lace- or web-like rash, bumps under the skin,
and abdominal pain after eating.
Researchers believe that this type of vasculitis is
very rare, although the symptoms can be similar to those of other types of
vasculitis. Some cases of polyarteritis nodosa seem to be linked to hepatitis B
or C infections.
Mostly Small Vessel Vasculitis
These types of vasculitis usually, but not always,
affect the small blood vessels.
Churg-Strauss Syndrome
Churg-Strauss syndrome is a very rare disorder that
causes blood vessel inflammation. It's also known as allergic angiitis and
granulomatosis (GRAN-u-lo-ma-TO-sis).
This disorder can affect many organs, including the
lungs, skin, kidneys, nervous system, and heart. Symptoms can vary widely. They
may include
asthma,
higher than normal levels of white blood cells in the blood and tissues, and
tissue formations known as granulomas.
Essential Mixed Cryoglobulinemia
Essential mixed cryoglobulinemia (KRI-o-GLOB-u-luh-NE-me-uh)
can occur alone, or it may be linked to a systemic vasculitis. A systemic
vasculitis is one that affects the body in a general or overall way.
Symptoms often include joint aches; weakness; nerve
changes, such as numbness, tingling, and pain in the limbs; kidney
inflammation; and a raised, bumpy, reddish-purple skin rash known as palpable
purpura (PURR-purr-ah).
While essential mixed cryoglobulinemia can occur
with other conditions, it most often is linked to chronic hepatitis C
infection.
Henoch-Schönlein Purpura
Henoch-Schönlein purpura (HSP) is a type of vasculitis
that affects the smallest blood vesselsthe capillariesin the skin,
joints, intestines, and kidneys.
Symptoms often include abdominal pain, aching and
swollen joints, and signs of kidney damage, such as blood in the urine. Another
symptom is a bruise-like rash that mostly shows up as reddish-purple blotches
on the lower legs and buttocks (although it can appear anywhere on the
body).
HSP is more common in children, but it also can
affect teens and adults. In children, about half of all cases follow a viral or
bacterial upper respiratory infection. Most people get better in a few weeks
and have no lasting problems.
Hypersensitivity Vasculitis
Hypersensitivity vasculitis affects the skin. This
condition also is known as allergic vasculitis, cutaneous vasculitis, or
leukocytoclastic vasculitis.
A common symptom is red spots on the skin, usually
on the lower legs. For people who are bedridden, the rash appears on the lower
back.
An allergic reaction to a medicine or infection
often causes this type of vasculitis. Stopping the medicine or treating the
infection usually clears up the vasculitis. However, some people may need to
take anti-inflammatory medicines, such as corticosteroids, for a short time.
These medicines help reduce inflammation.
Microscopic Polyangiitis
Microscopic polyangiitis (POL-e-an-je-I-tis) affects
small vessels, particularly those in the kidneys and the lungs. This disease
mainly occurs in middle-aged people; it affects men slightly more often than
women.
The symptoms often aren't specific, and they can
begin gradually with fever, weight loss, and muscle aches. In some cases, the
symptoms come on suddenly and progress quickly, leading to kidney failure.
If the lungs are involved, the first symptom may be
coughing up blood. In some cases, the disease occurs along with a vasculitis
that affects the intestinal tract, the skin, and the nervous system.
The signs and symptoms of microscopic polyangiitis
are similar to those of the vasculitis condition called Wegener's
granulomatosis. However, microscopic polyangiitis usually doesn't affect the
nose and sinuses or cause abnormal tissue formations in the lungs and
kidneys.
The results of certain
blood
tests can suggest inflammation. These results include a higher than normal
erythrocyte sedimentation rate (ESR); lower than normal hemoglobin and
hematocrit levels (which suggest anemia); and higher than normal white blood
cell and platelet counts.
Also, more than half of the people with microscopic
polyangiitis have certain antibodies called antineutrophil cytoplasmic
autoantibodies (ANCA) in their blood. These antibodies also are seen in people
who have Wegener's granulomatosis.
Testing for ANCA can't be used to diagnose either of
these two types of vasculitis. However, testing can help evaluate people who
have vasculitis-like symptoms.
Wegener's Granulomatosis
Wegener's granulomatosis is a rare vasculitis. It affects men
and women equally, but occurs more often in Whites than in African Americans.
This type of vasculitis can occur at any age, but it is more common in
middle-aged people.
Wegener's granulomatosis typically affects the
sinuses, nose, and throat; the lungs; and the kidneys. Other organs also can be
involved.
In addition to inflamed blood vessels, the affected
tissues also develop abnormal formations called granulomas. If granulomas
develop in the lungs, they can destroy the lung tissue. The damage can be
mistaken for
pneumonia
or even lung cancer.
Symptoms of Wegener's granulomatosis often are not
specific and can begin gradually with fever, weight loss, and muscle aches.
Sometimes, the symptoms come on suddenly and progress rapidly, leading to
kidney failure. If the lungs are involved, the first symptom may be coughing up
blood.
The results of certain blood tests can suggest
inflammation. These results include a higher than normal ESR; lower than normal
hemoglobin and hematocrit levels (which suggest anemia); and higher than normal
white blood cell and platelet counts.
Another test looks for antiproteinase-3 (an
antineutrophil cytoplasmic autoantibody) in the blood. Most people who have
active Wegener's granulomatosis will have this antibody. A small portion may
have another ANCA known as antimyeloperoxidase-specific ANCA.
Having either ANCA antibody isn't enough on its own
to make a diagnosis of Wegener's granulomatosis. However, testing for the
antibodies can help support the diagnosis in patients who have other signs and
symptoms of the condition.
A biopsy of an affected organ is the best way for
your doctor to make a firm diagnosis. A biopsy is a procedure in which your
doctor takes a small sample of your body tissue to examine under a
microscope. |