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What is vasculitis?
Vasculitis is a general term for a group of uncommon diseases that
feature inflammation of the blood vessels. The blood vessels of the
body are referred to as the vascular system. The blood vessels are
composed of arteries that pass oxygen-rich blood to the tissues of the
body and veins that return oxygen-depleted blood from the tissues to
the lungs for oxygen. Vasculitis is characterized by inflammation in
and damage to the walls of various blood vessels.
Each of the vasculitis diseases (also, as a group, referred to as
vasculitides) is defined by certain patterns of distribution of blood
vessel involvement, particular organ involvement, and laboratory test
abnormalities.
The word vasculitis is derived from the Latin "vasculum", vessel + "itis", inflammation. Another term for vasculitis is angiitis. When
arteries are the inflamed blood vessels, the condition is also referred
to as arteritis. When the veins are inflamed, it is referred to as
venulitis.
Vasculitis is an inflammation of the blood vessels in the body. In
vasculitis, the body’s immune system mistakenly attacks the body’s
own blood vessels, causing them to become inflamed. Inflammation
can damage the blood vessels and lead to a number of serious
complications.
Vasculitis can affect any of the body’s blood vessels. These include
arteries, veins, and capillaries. Arteries are vessels that carry blood
from the heart to the body’s organs, veins are the vessels that carry
blood back to the heart, and capillaries are the tiny blood vessels that
connect the small arteries and veins.
When a blood vessel becomes inflamed, it can:
•
Narrow, making it more difficult for blood to get through
Close off completely so that blood can’t get through at all (occlusion)
• In rare cases, stretch and weaken so much that it bulges
(aneurysm) and may possibly burst (aneurysm rupture)
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Figure A shows a normal artery with normal blood flow (the inset
image shows a cross-section of the normal artery). Figure B shows an
artery narrowed due to inflammation in the arterial wall, causing
decreased blood flow (the inset image shows a cross-section of the
inflamed artery). Figure C shows a totally occluded (blocked) artery
due to inflammation and scarring in the arterial wall (the inset image
shows a cross-section of the block artery). Figure D shows an artery
containing an aneurysm.
The disruption in blood flow from inflammation can damage the
body’s organs. Specific signs and symptoms depend on which organ
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has been damaged and the extent of the damage. Typical signs and
symptoms of inflammation (fever, swelling, and a general sense of
feeling ill) are common among people with vasculitis.
What are the symptoms of Vasculitis?
The symptoms of vasculitis depend on the particular blood vessels
Those are involved by the inflammatory process. Different types of
vasculitis involve blood vessels in characteristic locations throughout
the body. For example, Giant Cell Arteritis typically involves the
medium– to large–sized blood vessels supplying the head and neck,
but rarely involves the blood vessels of the kidneys. In contrast,
Wegener’s Granulomatosis frequently involves the kidneys, very
often the lungs, and almost always the upper respiratory tract, but
rarely blood vessels to the brain. As depicted in the image below,
Buerger's disease involves the fingers and (toes). Gangrene can result
from a profound lack of blood flow.
Different types of vasculitis have characteristic (localized) patterns of
blood vessel involvement. However, vasculitis is a systemic illness.
Thus, patients with vasculitis feel sick. They often have fevers,
weight loss, fatigue, a rapid pulse, and diffuse aches and pains that are
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difficult to pinpoint. It has been said that vasculitis is a “hurting
disease”, because it is so commonly associated with pain of one type
or another: pain from a nerve infarction, pain from insufficient blood
to the gastrointestinal tract, pain from skin ulcers. In some cases,
however, identifying the source and underlying cause of the pain is
extremely challenging. In addition to these diffuse, poorly–localized
"constitutional symptoms", vasculitis may involve virtually every
organ system in the body
What Organ Systems are affected?
Joints
Lungs
Kidneys
Gastrointestinal tract
Blood
Sinuses, nose, and ears
Eyes
Brain
Nerve
A variety of rashes, the most classic of which is "palpable purpura" –
purplish–red spots, usually found on the legs. These spots can usually
be felt by the examiner's fingertips, hence the descriptor "palpable".
The image below is a classic example of palpable purpura. These
lesions result from the leakage of blood into the skin through
inflamed, damaged blood vessels. They tend to occur in "crops"
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Repeated bouts of purpura may lead to hyper-pigmented areas of the
skin, as shown below
Joints
Symptoms range from full–blown arthritis to aches in the joints
without obvious swelling (arthralgias)
Pictured below is an example of Henoch-Schönlein purpura:
cutaneous vasculitis manifested by palpable purpura and arthritis
(note the right ankle swelling). The diagnosis was confirmed by a skin
biopsy, with immunofluorescence positive for IgA deposition within
blood vessel walls
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Lungs
Cough (particularly coughing up blood), shortness of breath, a
pneumonia–like appearance to a patient's chest X–ray, lung
"infiltrates", and the development of cavities in the lungs.
Below is an image of a CAT scan of the lungs of a 73 year–old
woman complaining of constitutional symptoms, shortness of
breath, and bloody sputum. The patient also had
glomerulonephritis, a positive P–ANCA, and antibodies to
myeloperoxidase. The diagnosis of microscopic polyangiitis was
made.
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Eleven days later, as the patient's symptoms worsened, a chest X–ray
confirmed progression of her lung hemorrhage. The X–ray shows
fluffy infiltrates in both lungs, representing bleeding from damaged
capillaries.
Kidneys
Red blood cells (usually invisible to the naked eye), clumps of red
blood cells (known as “casts”, also invisible to the naked eye), and
loss of protein in the urine. May lead to renal insufficiency and the
requirement of dialysis. Depicted in the figure below is a single
glomerulus (the filtering unit of the kidneys; each kidney has
approximately 1 million glomeruli). This glomerulus is involved
actively by an inflammatory process, particularly evident in the
bottom of the figure.
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Gastrointestinal Tract
Abdominal pain, bloody diarrhea, perforation of the intestines.
Blood
Anemia (low hematocrit or red blood cell count) and/or a slightly
elevated white blood cell count.
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Sinuses, Nose, and Ears
Chronic sinus congestion and “infections” that persist for longer
than they should; hearing loss; inflammation of the nasal septum,
sometimes resulting in a perforation or collapse of the bridge of the
nose, as shown in the picture below.
Eyes
May affect either blood vessels to the eyes, causing the sudden loss of
vision, or small blood vessels within the eyes, leading to retinal
problems, thinning of the sclera (the white part of the eyes),
inflammation within the eye's different chambers, and conjunctivitis
(“pinkeye”). Pictured below is an example of retinal vasculitis in a
patient with systemic lupus erythematosus (lupus). The white areas
represent regions of retinal infarction caused by vasculitis.
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Brain
Headaches, strokes, changes in mental status, difficulty with
coordination. Below, a magnetic resonance (MR) imaging study of
the brain in central nervous system vasculitis demonstrates an intra–
cerebral hemorrhage (bright area).
Nerve
Shooting pains in the arms and legs, numbness, and asymmetrical
weakness (i.e., weakness that involves one side of the body more than
the other).
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Signs and symptoms
The signs and symptoms of vasculitis vary depending on which blood
vessels and, as a result, which organ systems are affected. However,
general signs and symptoms that most people with vasculitis
experience include
Fever
Fatigue
Weight loss
Muscle and joint pain
Loss of appetite
Nerve problems, such as numbness or weakness
Each type of vasculitis can also cause specific signs and symptoms,
such as:
Behcet's syndrome. This condition causes inflammation of your
arteries and veins, and often appears in your 20s and 30s. Signs and
symptoms include mouth and genital ulcers, eye inflammation and
acne-like lesions on your skin.
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Buerger's disease. Also called thromboangiitis obliterans, this
condition causes inflammation and clots in the blood vessels in
your extremities. Signs and symptoms can include pain in your
hands, arms, feet and legs, and ulcers on your fingers and toes. This
disorder is strongly associated with cigarette smoking.
Churg-Strauss syndrome. This condition, also known as allergic
granulomatosis and angiitis, most commonly affects the blood
vessels in your lungs. It's often associated with asthma.
Cryoglobulinemia. This condition is often associated with
hepatitis C infections. Signs and symptoms include a hemorrhagic
rash (purpura) on your lower extremities, arthritis, weakness and
nerve damage (neuropathy).
Giant cell arteritis (GCA). This condition, which usually occurs in
people older than 50, is an inflammation of the arteries in your
neck, upper body, arms, and — most often — head, especially your
temples. GCA can cause headaches, scalp tenderness, jaw pain,
blurred or double vision, and even blindness. GCA is often
associated with polymyalgia rheumatica.
Henoch-Schonlein purpura. This condition is caused by
inflammation of the blood vessels of your skin, joints, bowel and
kidneys. Signs and symptoms can include abdominal pain, blood in
the urine or stool, joint pain, and a hemorrhagic rash (purpura) on
your buttocks, legs and feet. Henoch-Schonlein most often occurs
in children, but it can occur at any age.
Hypersensitivity vasculitis. The primary sign of hypersensitivity
vasculitis is red spots on your skin. It can be triggered by an allergy,
most often to a medication or an infection.
Kawasaki disease. Also known as mucocutaneous lymph node
syndrome, this condition most often affects children younger than 5
years of age. Signs and symptoms include fever, skin rash and eye
inflammation.
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Microscopic polyangiitis. This form of vasculitis affects smallsized blood vessels in your kidneys, lungs and skin. Signs and
symptoms include skin lesions, fever, unintentional weight loss,
glomerulonephritis — inflammation of the small blood vessels in
the kidneys — and nerve damage.
Polyarteritis nodosa. This form of vasculitis affects small- to
medium-sized blood vessels in many different parts of the
body, including your skin, heart, kidneys, peripheral nerves,
muscles and intestines. Signs and symptoms include purpura,
skin ulcers, muscle and joint pain, abdominal pain, and kidney
problems.
Polymyalgia rheumatica (PMR). This condition primarily affects
older adults and results in pain and inflammation of the large
joints, such as your shoulders, hips and knees. Signs and
symptoms include pain and stiffness in the muscles of your
hips, thighs, shoulders, upper arms and neck. PMR often
occurs in association with giant cell arteritis.
Rheumatoid vasculitis. This type of vasculitis can complicate the
course of rheumatoid arthritis and usually occurs in people
with a history of severe rheumatoid arthritis. Many different
parts of the body, including the eyes, skin, hands and feet may
be involved.
Takayasu's arteritis. This form of vasculitis includes the largest
arteries in the body, including the aorta, and typically occurs
in young women. Signs and symptoms include back pain, arm
weakness or pain with use (claudication), decreased or absent
pulses, lightheadedness, headaches, and visual disturbances.
Wegener's granulomatosis. This condition causes inflammation of
the blood vessels in your nose, sinuses and throat (collectively called
the upper respiratory tract), lungs, and kidneys. Signs and symptoms
can include shortness of breath, nasal stuffiness, chronic sinusitis,
nosebleeds and frequent ear infections.
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What causes vasculitis and what are examples of diseases with
vasculitis?
The actual cause of these vasculitis diseases is usually not known.
However, immune system abnormality and inflammation of blood
vessels are common features. Each form of vasculitis has its own
characteristic pattern of symptoms. Vasculitis can also accompany
infections (such as hepatitis B), exposure to chemicals (such as
amphetamines and cocaine), medications, cancers (such as
lymphomas and multiple myeloma), and rheumatic diseases (such as
rheumatoid arthritis, systemic lupus erythematosus).
Vasculitis is an inflammation of the blood vessels, but what causes
the inflammation is often unknown. It is sometimes a side effect of
the body’s immune system response to a recent or chronic infection.
It also can be a side effect of the body’s response to a medicine. The
body sometimes recognizes a medicine as a foreign substance, and
may develop an “allergic reaction” to try to get rid of it.
Vasculitis is sometimes linked to other diseases, such as:
Autoimmune system diseases. These are diseases that the vasculitis
patient may have had for years, in which the body’s immune system
mistakenly attacks the body itself. Examples include systemic lupus
erythematosus (lupus), rheumatoid arthritis, and scleroderma.
Blood cancers. These include leukemia and lymphoma
The causes of most vasculitides are currently unknown. It is clear,
however, that the immune system plays a critical role in the tissue
damage caused by vasculitis. The immune system, normally a
protective organ of the body, becomes “hyperactive” in vasculitis
because of some unknown stimulus, leading to inflammation within
the body’s tissues. Inflammation in blood vessel walls leads to
narrowing of the vessels the resulting inadequate blood supply to a
particular tissue or organ results in damage.
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Your vascular system is an intricate network of blood vessels,
including veins, arteries and capillaries. Systemic arteries deliver
oxygen-rich blood to your body's tissues, while veins return blood
with increased amounts of carbon dioxide — a waste product of
metabolism — to your heart. Capillaries, the smallest blood vessels,
connect the veins and the arteries and permit the transfer of fluids
and nutrients to and from the surrounding tissues.
In vasculitis, the blood vessels become inflamed, which can cause the
layers of the blood vessel wall to thicken. This narrows the blood
vessels, reducing the amount of blood — and therefore oxygen and
vital nutrients — that reaches your body's tissues. In some cases, a
blood clot may form in an affected blood vessel, obstructing blood
flow. Sometimes instead of becoming narrower, a blood vessel may
weaken and form a bulge (aneurysm), a potentially life-threatening
condition.
Primary vs. secondary
For many of its forms, the cause of vasculitis is unknown. These
forms of vasculitis are called primary vasculitides.
For some types, however, infections may be the cause. Forms of
vasculitis for which an underlying disease is the cause are called
secondary vasculitides. For instance, most cases of cryoglobulinemia
are the result of the hepatitis C virus infection, and the hepatitis B
virus infection causes some cases of polyarteritis nodosa. Vasculitis
can also occur as the result of some diseases of the immune system,
such as rheumatoid arthritis, lupus and Sjogren's syndrome.
Sometimes an allergic reaction to a medication, such as an antibiotic
or diuretic, may cause vasculitis.
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How is vasculitis diagnosed?
Laboratory testing of blood or body fluids in a patient with active
vasculitis generally indicates inflammation in the body. Depending on
the degree of organ involvement, a variety of organ function tests can
be abnormal.
The diagnosis of vasculitis is definitively established after a biopsy of
involved tissue demonstrates the pattern of blood vessel
inflammation. Examples of tissues used for biopsy include skin,
sinuses, lung, nerve, and kidney. Depending upon the situation, an
alternative to biopsy can be an x-ray test of the blood vessels called
an angiogram, which can demonstrate characteristic patterns of
inflammation.
The diagnosis of vasculitis is based on a person’s medical history,
physical exam, signs and symptoms, and laboratory tests.
How do we diagnose Vasculitis?
Patients with vasculitis learn that making the diagnosis is sometimes
quite difficult. Many endure numerous doctors’ visits, tests, and
hospitalizations before the pieces of the puzzle are assembled. The
diagnosis of vasculitis usually requires a biopsy of an involved organ
to secure a "tissue diagnosis". The two reasons for this are:
# ONE: Vasculitis has many MIMICKERS (other diseases that
have similar features but require different treatments).
# TWO: The treatments for vasculitis itself involve substantial risk.
No
physician should prescribe such treatment without making every
effort to secure a firm diagnosis.
Blood tests, X–rays, and other studies may suggest the diagnosis of
vasculitis, but often the only way to clinch the diagnosis is to biopsy
an involved tissue, examine the tissue under the microscope in
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consultation with a pathologist (ideally one experienced at examining
biopsies in vasculitis), and find the pathologic hallmarks of the
disease. The trick to diagnosing vasculitis is to figure out the least
invasive method of accomplishing this task. If a patient’s symptoms,
physical examination, and diagnostic testing suggest involvement of a
particular organ, one of the procedures below may be used to
confirm (or exclude) the diagnosis of vasculitis:
(Top of page)
Skin Biopsy
One of the least invasive ways of making the diagnosis. A minor
procedure performed under local anesthesia. The wound is closed
with 1–2 stitches that are removed 7–10 days later. Pictured below
is an abnormal skin biopsy showing leukocytoclastic vasculitis. The
white oval shapes are subcutaneous fat cells beneath the dermis.
Pictured below is an example of an inadequate skin biopsy
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The correct diagnosis of PAN (polyarteritis nodosa) was not
confirmed by this biopsy because the biopsy was not deep enough.
The biopsy specimen contains only the epidermis and superficial
dermis. PAN classically affects medium–sized arteries located in the
deep dermis.
In contrast to the biopsy above, the skin biopsy below was deep
enough to include the deep dermis as well as some subcutaneous fat.
The white, oval–shaped areas are fat lobules. Just superficial to the
subcutaneous fat, within the deep dermis, an inflamed medium–sized
vessel is evident. A closer view of the vessel is provided in the next
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figure which provides a high power view of the vasculitic artery lying
at the junction of the dermis and subcutaneous fat.
Kidney Biopsy
Performed if there is evidence of kidney involvement by vasculitis
(red blood cells or protein in the urine, for example). Done under
local anesthesia while the kidney is visualized by ultrasound. Because
of the small but significant risk of bleeding after this procedure,
patients are usually monitored in the hospital for 24 hours after the
biopsy. The biopsy below shows a “crescent” in a glomerulus.
Sural Nerve Biopsy
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The sural nerve is a sensory nerve over the lateral aspect of the foot.
Under local anesthesia in an operating room, a surgeon removes a
small piece of the nerve.
usually along with a piece of the adjacent muscle (the gastrocnemius).
Because the sural nerve does not innervate muscles (remember: it is a
sensory nerve, not a motor nerve), the patient does not lose any
strength on the side of the foot and lower leg. There maybe,
however, some residual numbness on the side of the foot. Patients
generally tolerate this numbness well (if the vasculitis has involved
the nerve severely enough, some patients already have numbness in
that region). Below is the surgical site of a sural nerve and
gastrocnemius muscle biopsy one week after the procedure: a few
sutures and a thin, well–healing scar.
Temporal Artery Biopsy
Performed to diagnose Giant Cell Arteritis, also known as Temporal
Arteritis, because the temporal artery is often involved. The temporal
artery courses up the temples, just in front of the ears. The biopsy,
done under local anesthesia, is performed by making a small incision
just above the hairline (sometimes shaving a small area of hair is
required). The procedure is extremely well–tolerated by patients.
Within several weeks, there is usually little or no sign that a biopsy
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was done. Complications of temporal artery biopsies are extremely
rare. Sometimes, to increase the diagnostic yield, both temporal
arteries (i.e., the ones on each side of the head) are biopsies.
Lung Biopsy
Often the best way to make a diagnosis of vasculitis that involves the
lungs, such as Wegener’s Granulomatosis. May be performed in one
of two ways: 1) open lung biopsy, a sizeable surgical procedure; or 2)
thoracoscopic lung biopsy, a less invasive but still significant
procedure. Even a thoracoscopic biopsy usually requires at least 48
hours in the hospital and the temporary placement of a chest tube to
permit the lung to re–expand.
Brain Biopsy
Often necessary to confirm the diagnosis of Central Nervous System
(CNS) Vasculitis. Usually performed on the non–dominant side of
the patient’s brain (that is, if the patient is right–handed — and
therefore “left–brained” — the biopsy is performed on the right side
of the brain). Biopsy of the brain’s covering, the meninges, is usually
performed at the same time.
Abdominal Angiogram
Helpful in the diagnosis of Polyarteritis Nodosa (PAN). Similar to a
heart catheterization. After inserting a catheter into a large artery in
the leg and advancing the catheter into the aorta, radiographic dye is
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injected into blood vessels supplying the gastrointestinal tract. In the
proper clinical setting, the detection of aneurysms (small
outsourcings of blood vessel walls) is diagnostic of PAN.
Central nervous system angiogram
Frequently part of the “work–up” of CNS vasculitis. The procedure
is identical to an abdominal angiogram, except the catheter is
advanced all the way up to the large vessels supplying the head and
neck (for example, the carotid arteries). On angiography, CNS
vasculitis is characterized by “beading” (dilated areas alternating with
narrowing of the blood vessels). A strikingly abnormal angiogram
may eliminate the need for a brain biopsy. The angiogram pictured
below shows prominent dilations of arteries visible at several sites in
the intra–cerebral region.
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Other Useful Tests
There are many other tests that are helpful in the diagnosis of
vasculitis, or in evaluating the activity of the disease.
Erythrocyte sedimentation rate (ESR)
C–reactive protein (CRP)
Urinalysis
CT Scan
ANCA tests
Erythrocyte sedimentation rate (ESR) – Also known as the “sed
rate”, for short. This is an old but useful test first employed by the
ancient Greeks as a test for pregnancy (the ESR is elevated not only
in inflammatory conditions, but also in pregnancy).
C–reactive protein (CRP) — A modern day version of the ESR,
more expensive but not necessarily superior to the ESR in the
diagnosis and management of vasculitis. CRP is a protein produced
by the liver in response to inflammation within the body.
Urinalysis — Many forms of vasculitis affect the kidneys. A simple
way of determining whether or not the kidneys are involved is to
perform a urinalysis. By performing checks for several indicators of
inflammation in a patient's urine, the physician may determine if
inflammation is present within the kidneys. These indicators include:
•
•
•
Protein (“proteinuria”)
Red blood cells (“hematuria”)
Clumps of red blood cells (“casts”)
Pictured below is a urine specimen from a patient with Wegener's
granulomatosis and glomerulonephritis (inflammation in the kidneys).
This is a view of the specimen examined under the microscope,
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showing cylindrical “casts” comprised of red blood cells. This finding
strongly indicates vasculitis in the kidney.
From another Wegener's granulomatosis patient's urinalysis, "blebs"
(identified by white arrows) protrude from the surface of the red
blood cells that have been damaged in transit through the kidney.
Because inflamed kidneys leak blood, red blood cells -- dismorphic as
these are -- appear in the urine. (pictured below)
CT scan (a CAT scan, or computed tomography) — A type of
radiology test that permits a non-invasive, cross–sectional view of a
patient's anatomy. On the illustration below (a chest CT scan from a
patient with Wegener's granulomatosis), the view is up (looking
toward the patient's head, from his or her feet). The heart is the
white, rounded object in the upper center of the picture.
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The black regions are the patient's lungs. The large spot in the left
lung (corresponding to the patient's right lung) is a nodule caused by
Wegener's. Other smaller nodules are also evident.
ANCA tests — ANCA is an abbreviation (acronym) for anti–
neutrophil cytoplasmic antibodies. These antibodies are found in the
blood of patients with several different types of vasculitis, including
Wegener's Granulomatosis, Microscopic Polyangiitis, and the Churg–
Strauss Syndrome. ANCAs and their association with vasculitis were
recognized in the mid–1980s, and their use has become increasingly
widespread since the 1990s. ANCAs are detected by a simple blood
test. These antibodies are directed against the cytoplasm (the non–
nucleus part) of white blood cells. Their precise role in the disease
process remains uncertain but is a topic of considerable research
interest. ANCAs come in two primary forms: 1) the C–ANCA [C
stands for cytoplasmic] and, 2) the P–ANCA [P stands for
perinuclear]. C–ANCAs have a particularly strong connection to
Wegener’s Granulomatosis (up to 80% of patients – and possibly
more of those with active disease – have these antibodies). When C–
ANCAs are present in the blood of a patient with symptoms or signs
suggesting Wegener’s, the likelihood of the diagnosis increases
considerably. Because of the long list of other conditions that are
sometimes associated with ANCAs, however, in most cases it is still
VERY IMPORTANT to biopsy an organ involved by vasculitis to
verify the diagnosis.
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Specialists Involved
A person with vasculitis may be referred to various specialists,
including
A dermatologist (skin specialist)
A hematologist (blood specialist)
A pulmonologist (lung specialist)
A cardiologist (heart specialist)
A neurologist (nervous system specialist)
An ophthalmologist (eye specialist)
A urologist (urinary tract and urogenital system specialist)
A nephrologist (kidney specialist)
An infectious disease specialist
Diagnostic Tests and Procedures
A variety of tests are used to diagnose vasculitis. The type of test
chosen will depend on the signs and symptoms a person has. Some
of the tests used in the diagnosis of vasculitis include:
Blood tests. These may be done to look for abnormal levels of blood
cells or antibodies, which could be a sign of inflammation in the
body.
Biopsy. During this test, the doctor takes a small sample of tissue
from a blood vessel or one of the affected organs and looks at it
under a microscope for inflammation or damage. A biopsy is often
the best way to make a firm diagnosis of vasculitis.
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Urine analysis. This test might be done to look for abnormal levels of
protein or blood cells in the urine, which could be a sign of vasculitis
affecting the kidneys.
EKG (electrocardiogram). This test measures the rate and regularity
of the heartbeat, and is done to see if vasculitis is affecting the heart.
Echocardiogram. This test uses sound waves to create a moving
picture of your heart. Echocardiogram provides information about
the size and shape of your heart and how well your heart chambers
and valves are functioning. The test also can identify areas of poor
blood flow to the heart, areas of heart muscle that are not contracting
normally, and previous injury to the heart muscle caused by poor
blood flow.
Chest x ray. A chest x ray takes a picture of the organs and structures
inside the chest, including the heart, lungs, and blood vessels. A chest
x ray may show abnormal changes if vasculitis is affecting the lungs.
Pulmonary function testing. These are breathing tests that evaluate
how well the lungs are working. These tests are done to see if
vasculitis is affecting how the lungs work.
Abdominal ultrasound. This test uses sound waves to create a picture
to look for vasculitis affecting the abdominal organs. It is similar to
an echocardiogram.
Computerized tomography (CT) scan. A CT scan provides a
computer generated x-ray image of the internal organs. CT scans can
be used to look for vasculitis affecting the abdominal organs or the
brain.
Magnetic resonance imaging (MRI). This test uses powerful magnets
and radio waves to make images and can be used to look for a
vasculitis affecting the brain.
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Angiography. This test may be done to see the flow of blood through
the blood vessels and to determine whether they are blocked. During
this test, a dye is injected into the blood vessels, and x-ray pictures of
the blood vessels are taken
Type of
Test
Complete
Blood
Count
(“CBC”)
What should be
checked
• White blood cells
(WBC)
• Platelets
• Hematocrit
Why?
•
•
•
Low WBC count may
lead to infections.
Low platelets may
cause bleeding.
Low hematocrit
means insufficient
oxygen-carrying
capacity of the blood.
Kidney
Function
•
•
Creatinine
Blood Urea
Nitrogen (BUN)
•
High creatinine and
BUN indicate that the
kidneys are not
performing their
blood-cleansing
function properly.
Urinalysis
•
•
Protein Level
Red Blood Cells
•
Normal urinalyses
have no protein and
no blood.
The presence of
protein and/or blood
in the urine may
indicate active
vasculitis in the
kidneys (or damage
to the bladder from
cyclophosphamide).
•
Liver
Function
•
•
•
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Albumin
Aspartate
aminotransferas
e(AST)
Alanine
aminotransferas
e (ALT)
•
•
Often a good
indication of overall
health.
Elevated AST/ALT
levels indicate
inflammation in the
liver (usually caused
by medications).
What Are the Signs and Symptoms of Vasculitis?
Vasculitis can have many different signs and symptoms depending on
the type of vasculitis and which organs are involved as well as how
severely they are affected. With vasculitis, any number of organs may
be involved, so a patient can experience very few signs and symptoms
or may be very sick.
There are two types of signs and symptoms that are common among
people with vasculitis: those that affect the body in a general way
(systemic) and those that affect specific organs or body systems.
Systemic
Systemic signs and symptoms are not specific to a particular part of
the body, but affect a person overall, causing general aches, pains,
and sickness. Common systemic symptoms include:
Fever
Loss of appetite
Weight loss
Fatigue (feeling tired) and weakness
General aches and pains
Organ- or Body System-Specific
These signs and symptoms are specific to a particular organ or body
system. The organs and body systems that can be affected include:
Skin. People may experience a variety of skin changes, including
purple or red spots. The changes may look like clusters of small dots,
splotches, bruises, or hives. They may be itchy or painless.
Joints. People can experience aches and arthritis if the joints are
affected.
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Lungs. People may experience shortness of breath or even cough up
blood. On a chest x ray, lung symptoms may look like pneumonia,
even though they are not.
Gastrointestinal tract. Ulcers in the mouth may be present in some
types of vasculitis. Also, abdominal pain or bloody diarrhea can occur
in people with vasculitis. In some severe cases, the intestines can
rupture.
Sinuses, nose, and ears. People may experience sinus infections,
chronic middle ear infections, ulcers in the nose, or in certain cases
there may be hearing loss.
Eyes. People whose eyes are affected by vasculitis may experience a
blurring or loss of vision.
Brain. People may experience headaches, confusion, changes in
behavior, or strokes.
Nerves. People may experience numbness, tingling, and weakness in
various parts of the body. They also may experience symptoms in
their limbs, such as loss of feeling or strength in the hands and feet or
shooting pains in the arms and legs.
How is vasculitis treated?
Treatment
Specific treatment for vasculitis depends on your type of vasculitis,
the severity of your case and your general health. Your doctor will ask
if you've had any past reactions to medications, nutritional
supplements and sun exposure, all of which may bring on allergic
skin rashes in some people.
Though some types of vasculitis are self-limiting and improve on
their own, such as Henoch-Schonlein purpura, others involve taking
one or more of the following medications.
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Corticosteroids. Treatment for many types of vasculitis consists of
doses of a corticosteroid drug, such as prednisone or
methylprednisolone (Medrol). You often start feeling better in just a
few days, but you may need to continue taking medication for an
extended period of time. After the first month, your doctor may
gradually begin to lower the dose until you reach the lowest dose of
corticosteroids you need to control inflammation.
Some of your signs and symptoms may return during this tapering
period.
Your doctor may also have you take medications to combat the side
effects of long-term treatment with corticosteroids. For instance, the
bisphosphonates risedronate (Actonel) and alendronate (Fosamax)
can treat steroid-induced osteoporosis. Risedronate is also approved
for the prevention of osteoporosis.
Cytotoxic drugs. Some cases of vasculitis that are severe or that
don't respond well to corticosteroids may need treatment with
cytotoxic drugs, such as azathioprine (Imuran) and
cyclophosphamide (Cytoxan). These drugs suppress the inflammation
in your blood vessels.
Nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs, such
as aspirin and ibuprofen (Advil, Motrin, others), can be effective in
treating mild symptoms of some types of vasculitis, such as
polymyalgia rheumatica or Kawasaki disease. But NSAIDs don't offer
complete relief for many people, and long-term use can cause
stomach and intestinal bleeding.
While you undergo treatment, your doctor may conduct periodic
blood tests to monitor your progress and response to treatment.
Other medications that researchers are studying in the treatment of
vasculitis include mycophenolate mofetil (CellCept), tumor necrosis
factor (TNF) blockers (Remicade, Enbrel) and rituximab (Rituxan).
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Because vasculitis is caused in part by an overly active immune
system, the treatment usually involves the use of medications that
suppress parts of the immune system. The most common treatments
of vasculitis are discussed below.
The vasculitic diseases are serious disorders, and often require
potentially dangerous treatments. For our readers’ benefit, we discuss
most of the potential side–effects of medications used in the
treatment of vasculitis. All of the medicines have a frighteningly long
list of possible side–effects, but we emphasize that many of the
side–effects can be avoided through careful management by
experienced physicians. The risk of some side–effects can be
reduced by taking supplementary medications along with those
specifically intended to treat vasculitis. Click below for more detailed
information on each medication.
The treatment of the various forms of vasculitis depends on the
severity of the illness and the organs involved. Treatments are
generally directed toward stopping the inflammation and suppressing
the immune system. Typically, cortisone-related medications, such as
prednisone, are used. Additionally, other immune suppression drugs,
such as cyclophosphamide (Cytoxan) and others, are considered.
Additionally, affected organs (such as the heart or lungs) may require
specific medical treatment when the disease is active.
The management of vasculitis is an evolving field in medicine. The
ideal programs for monitoring and treatment will continue to
improve as disease patterns and causes are defined by medical
research.
Most cases of vasculitis are treated with prescription medicines.
Goals of Treatment
The main goal of treatment is to stop the inflammation in the
affected blood vessels. Most treatments do this by stopping the
immune or inflammatory response that caused the vasculitis to occur.
Specific Types of Treatment
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There are two types of prescription medicines that are typically used
to treat vasculitis: corticosteroid medicines and cytotoxic medicines.
Corticosteroid Medicines
Corticosteroid medicines are often called steroids, though these are
not the same kind of steroids that athletes have been reported to use.
These medicines are used to reduce the inflammation in the blood
vessels. Examples of corticosteroids that the doctor might prescribe
include prednisone, prednisolone, and methylprednisolone.
Cytotoxic Medicines
Cytotoxic medicines are typically used to treat cancer, but certain
drugs also can be used to treat vasculitis. They may be prescribed in
severe cases or in cases in which the patient did not respond to
corticosteroids. Sometimes, they are prescribed along with
corticosteroids. Cytotoxic medicines work by killing the cells that
have caused the inflammation. Examples of these medicines include
azathioprine and cyclophosphamide. Doses used for vasculitis are
usually lower than those used to treat cancer
Vasculitis At A Glance
Vasculitis is a group of uncommon diseases which feature
inflammation of the blood vessels.
Diagnosis of vasculitis can be confirmed by a biopsy of
involved tissue or angiography.
Treatment is directed toward decreasing the inflammation of
the arteries and improving the function of affected organs
Types of Vasculitis
There are many different disorders that are classified as types of
vasculitis. Although all of these disorders include inflammation of the
blood vessels, most differ from each other in terms of what
populations of people and which organs they affect.
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Behcet’s Disease
Behcet’s disease can affect any part of the body because it can occur
in vessels of any size or type. Commonly affected organs include the
eyes, the mouth, and the genitals. Patients with this type of vasculitis
are often younger than those with other types, many times in their
twenties and thirties. The HLA-B51 gene allele is often associated
with Behcet’s disease, but not everyone with the gene will develop
the disease. Behcet’s is more common in individuals of
Mediterranean, Middle Eastern, or Far Eastern descent, but is usually
not found in Blacks.
Buerger’s Disease
Buerger’s disease typically affects blood flow to the hands and feet.
Buerger’s disease is also known as thromboangiitis obliterans and is
more likely to occur in smokers than nonsmokers.
Central Nervous System Vasculitis
Central nervous system vasculitis affects the brain and sometimes the
spinal cord.
Churg-Strauss Syndrome
Churg-Strauss syndrome can affect many different organs, but it
most commonly affects the lungs, skin, kidneys, and heart. Many
people who are diagnosed with Churg-Strauss syndrome also have
asthma. They may have had preexisting, newly diagnosed, or recently
worsened asthma when diagnosed with Churg-Strauss syndrome.
Cryoglobulinemia
Common signs and symptoms of cryoglobulinemia (KRI-o-GLOBu-luh-NE-me-uh) are a rash on the lower limbs, arthritis, and nerve
damage. Type 1 cryoglobulinemia is often associated with lymphoma,
while Type 2 cryoglobulinemia is often associated with hepatitis C
infection.
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Giant Cell Arteritis
Common symptoms of giant cell arteritis include headache, fever,
blurred vision, and pain in the jaw, shoulders, or hips. It is the most
common form of vasculitis that occurs in adults older than 50 years.
It is more likely to occur in people of Scandinavian origin, but can
occur in people of any race.
Henoch-Schonlein Purpura
Henoch-Schonlein purpura most commonly affects the skin, kidneys,
joints, and stomach. It is the most common form of vasculitis found
in children, but can occur at any age. When seen in children, it often
follows an upper respiratory infection. It is not usually a chronic
disease, and full recovery is common.
Microscopic Polyangiitis
Microscopic polyangiitis (POL-e-an-je-i-tis) most commonly affects
the kidneys, skin, and nerves, but it can affect any organ in the body.
Polyarteritis Nodosa
Polyarteritis nodosa (POL-e-ar-ter-i-tis no-DO-suh) most commonly
affects the kidneys, skin, and nerves, but it can affect any organ in the
body. It is most common in people in their thirties and forties. Men
are twice as likely as women to get polyarteritis nodosa. In some
cases, it is associated with chronic hepatitis B infection or a very
specific type of leukemia known as hairy cell leukemia.
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Polymyalgia Rheumatica
Polymyalgia rheumatica (POL-e-mi-AL-je-a ROO-ma-ti-kuh) most
commonly affects the large joints in the body, such as the shoulders
and hips. It often occurs along with giant cell arteritis.
Rheumatoid Vasculitis
Rheumatoid vasculitis can affect many different organs, including the
eyes, skin, hands, and feet. It commonly occurs in patients who have
severe rheumatoid arthritis.
Takayasu’s Arteritis
Takayasu (Tock-e-AH-sue) arteritis (ar-ter-I-tis) affects the aorta (the
largest blood vessel in the body) and its branches, which carry blood
away from the heart. It typically occurs in Asian women younger than
40.
Wegener’s Granulomatosis
Wegener’s granulomatosis (GRAN-u-lo-ma-TO-sis) can occur
anywhere in the body. It most commonly affects the upper
respiratory tract (nose, sinuses, and throat), lungs, and kidneys. It is
most common in people who are middle-aged and older
Other Names for Vasculitis
Angiitis
Arteritis
Who Is At Risk for Vasculitis?
Vasculitis can affect anyone, including men, women, and people of all
ages. Some types of vasculitis are more likely to affect certain
populations than others. Examples of populations that might be
more affected by certain types of vasculitis include smokers, children,
young women, middle-aged adults, and people with chronic hepatitis
B and/or C infections.
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How Can Vasculitis Be Prevented?
There is currently no known way to prevent vasculitis, but with
treatment, the complications of vasculitis can be prevented or
delayed.
Living with Vasculitis
General Information about Outcomes
The course of vasculitis is unpredictable and depends on the type and
severity of the disease. Vasculitis may:
Go into remission. If caught early it may respond well to treatment
and go into remission.
Reoccur. These reoccurrences are called flares, and they are generally
difficult to predict. Flares can sometimes happen when the doctor
takes the patient off of a medicine or alters the dose or type. Also,
certain types of vasculitis are more likely to flare than others, and
some patients are more likely to experience flares than others.
Remain chronic without remission. In these cases, vasculitis can
usually be controlled with continuing medicine treatments for an
extended period of time.
Not respond well to treatment. This can lead to disability or even
death. This is rare.
Ongoing Health Care Needs
The medicines used to treat vasculitis can have significant side
effects. Your doctor may adjust the type or dose of medicine you
take to lessen the side effects.
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In cases in which remission occurs, the doctor may carefully
withdraw medicines but still require careful monitoring of flares.
Patients who continue taking medicines should stay under the careful
watch of their doctor to monitor and control side effects. Patients
also should always monitor their health, side effects, and symptoms
and discuss any changes with their doctor.
Hypersensitivity Vasculitis
Hypersensitivity vasculitis (HV) is often used to describe different
types of vasculitis related to drug reactions, skin disorders or allergic
vasculitis; however this is not always the correct use of the term.
Given the wide range of symptoms, the varying definitions and
frequent incorrect use of the term, the American College of
Rheumatology made a list of criteria for the classification of HV.
Three or more of these criteria are needed to determine that a patient
with some form of vasculitis is defined as specifically having HV. The
criteria are:
(1) older than 16 years of age
(2) use of a drug before the development of symptoms
(3) skin rash
(4) biopsy of the skin rash that shows neutrophils, a type of white
blood cells, around a small vessel
It should be noted that having three of these criteria does not always
distinguish HV from other forms of vasculitis, particularly when the
only or first symptom of vasculitis is a skin rash.
The presence of skin vasculitis, usually red spots, is the main
symptom in hypersensitivity vasculitis. A biopsy of these skin spots
reveals inflammation of the small blood vessels, called a
leukocytoclastic vasculitis.
HV may be caused by a specific drug or occur in association with an
infection, but it may also be idiopathic, meaning there is no known
cause. Although drugs are the most common cause, drug-induced
vasculitis is a poorly defined disorder.
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There are no symptoms or tests that prove HV results directly from a
particular drug. The drugs that are most frequently listed as being
associated with the development of HV include: penicillin,
cephalosporin, sulfonamide, some medicines used to control blood
pressure (loop and thiazide-type diuretics), phenytoin and allopurinol.
Infections that may be associated with HV include hepatitis B or C
virus, chronic infection with bacteria and HIV virus (provide links?).
Symptoms: The major symptoms of HV, in addition to a skin rash,
are joint pains and increasing size of lymph nodes. Lymph nodes are
located in several places, but particularly along the neck, and supply
special cells to the bloodstream that help remove bacteria from the
body. In most patients, symptoms begin 7 to 10 days after the
exposure to the drug or infection, but can be as short as two to seven
days in some people.
Organ involvement in addition to the skin rash is very rare, but can
be severe. Kidney inflammation and even more rarely liver, lung,
heart and brain injury have occurred in patients with hypersensitivity
vasculitis. The kidney inflammation is usually mild.
Symptoms of kidney involvement may not be noticed by the patient,
but can be evaluated by a doctor by looking at a urine sample for
small amounts of blood and protein. Kidney failure is not common,
but can occur particularly with heavy or prolonged exposure to the
suspected drug or infection. Kidney failure can be ‘acute’, meaning
there is a fast loss of kidney function, but supportive treatment with
dialysis (mechanical cleansing of the blood) can be done for a few
days or weeks and kidney function returns. In some cases, ‘chronic’
kidney failure occurs, meaning that there is an ongoing need for
dialysis because the kidneys do not recover their normal function.
Treatment: If a drug may have caused the HV, then discontinuation
of that specific drug usually leads to the disappearance of symptoms
within a few days or weeks. If an infection may have caused the
hypersensitivity, then treatment of the infection usually results in the
disappearance of symptoms.
In some patients, especially those with ongoing infections such as
hepatitis B or C, there may be ongoing or ‘chronic’ symptoms of HV.
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Drugs used to manage the skin rash and joint pains associated with
HV might include corticosteroids and/or nonsteroidal antiinflammatory drugs.
In patients with more severe or ongoing skin rashes that are not due
to infection, drugs such as colchicine, antihistamines, and dapsone
(or a combination of these drugs) may be helpful to control
symptoms. Patients with disease in organs beyond the skin should be
referred to a specialty doctor such as a nephrologist if the kidneys are
involved.
Screening and diagnosis
The signs and symptoms of vasculitis resemble those of many
conditions, which can make a definite diagnosis difficult. As a result,
your doctor will try to rule out other possible causes of your
condition.
He or she will ask about your symptoms and past medical history and
conduct a thorough physical exam. You may also have some of the
following tests.
Blood tests. If your doctor suspects vasculitis, he or she may order a
blood test that checks your erythrocyte sedimentation rate —
commonly referred to as the sed rate. This test measures how quickly
red blood cells fall to the bottom of a tube of blood. Red cells that
drop rapidly may indicate inflammation in your body.
You also may have a test that measures C-reactive protein (CRP), a
substance produced by your liver in response to inflammation.
Your doctor may also check the number of red blood cells for
anemia and platelets (thrombocytes) in your blood. Platelets are
colorless blood cells that help stop blood loss when you're injured.
Some types of vasculitis result in an unusually high or low number of
these cells.
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Your doctor may also check to see if you have a high white blood
cell count, which can signify an infection or inflammation.
In addition, your doctor may check your blood for anti-neutrophil
cytoplasmic antibodies (ANCAs) and other antibodies, such as
rheumatoid factor (RF) and antinuclear antibody (ANA). ANCA
can indicate a diagnosis of Wegener's granulomatosis or microscopic
polyangiitis. RF and ANA elevations can be indications of an
associated rheumatoid arthritis or connective tissue disease.
Imaging studies. Your doctor may be able to determine whether
larger arteries, such as the aorta and its branches, are involved
through the use of noninvasive imaging techniques. These include
ultrasound, computerized tomography (CT) and magnetic resonance
imaging (MRI).
In some cases, though, you may need a more invasive X-ray test
called an angiogram. During this procedure, a catheter, resembling a
thin straw, is inserted into a large artery or vein. A special dye
(contrast medium) is then injected into the catheter, and X-rays are
taken as the dye fills these arteries or veins. The outlines of your
blood vessels are visible on the resulting X-rays.
Biopsy. Although blood tests and imaging studies can provide your
doctor with useful information, one of the best ways to confirm a
diagnosis of vasculitis is by taking a small sample (biopsy) of the
affected blood vessel. The procedure is performed on an outpatient
basis under local anesthesia, usually with very little discomfort or
scarring.
The sample is examined for signs of inflammation under a
microscope in a laboratory. If there is evidence of scarring, it implies
that the condition has been chronic, or long-standing. Treatment may
not be as effective in these cases, because the damage has been done
and may not be reversible.
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Urine test. This test may detect abnormalities, such as red blood
cells and increased amounts of protein, in your urine that often
indicate a medical problem. If the kidneys are involved, your
prognosis tends to be poorer.
Key Points
Vasculitis is an inflammation of blood vessels in the body. In
vasculitis, the body’s immune system mistakenly attacks the body’s
own blood vessels, causing them to become inflamed.
The inflammation can cause disruption of blood flow to parts of the
body, which can lead to tissue damage or even death.
Vasculitis can affect any blood vessel or organ in the body.
The exact cause of the inflammation in vasculitis is unknown, but it is
sometimes a side effect of the body’s normal immune system
response.
Vasculitis can affect people of all ages and either gender. Some types
of vasculitis are more likely to occur in certain populations than
others.
There are many types of vasculitis, and the signs and symptoms vary
widely in type and severity. They may be specific and affect a
particular organ, or they may be nonspecific, causing general aches,
pains, and fatigue (feeling tired).
Laboratory tests are usually performed to confirm a diagnosis of
vasculitis. There are many different tests that may be run, depending
on the signs and symptoms present and where they are in the body.
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Vasculitis is typically treated with prescription medicines with the
goal of stopping the inflammation in the blood vessels and relieving
symptoms.
Corticosteroid medicines or cytotoxic medicines are typically the
drugs used to treat vasculitis.
The course of a case of vasculitis is often difficult to predict.
Vasculitis may go into remission, reoccur, remain chronic, or, in rare
cases, lead to severe disability or death.
Frequently asked Questions
What causes vasculitis?
The causes of most forms of vasculitis remain unknown. Infections
are strongly suspected of playing a role in many forms, however. For
example, hepatitis B (a virus) causes some cases of polyarteritis
nodosa, and hepatitis C (another virus) is the cause of most cases of
cryoglobulinemia. Bacterial infections have been suspected of playing
a role in Wegener’s granulomatosis, and some patients with
Wegener’s that is limited to the upper respiratory tract are treated
only with an antibiotic, Bactrim (trimethoprim/sulfamethoxazole). A
general theory that applies to many types of vasculitis is that the
disease results from the occurrence of a particular infection in a
person whose genes (and other factors) make him/her susceptible to
developing vasculitis.
What is going to happen to me?
The course of vasculitis is often difficult to predict. Some types of
vasculitis tend to occur only once and not return. Other types are
prone to recurrences. For all patients with vasculitis, it is essential to
be evaluated by physicians experienced in the treatment of these
diseases. Most forms of vasculitis are treatable (see below), and many
patients may achieve remissions through treatment. The trick is often
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in balancing the types of medications necessary to control the disease
and the risk of side–effects that those medicines often bring. A
primary aim of several ongoing new studies in vasculitis is to find
drugs that help maintain remissions.
Is vasculitis curable?
Most forms of vasculitis are treatable if detected early enough,
before substantial organ damage has occurred. While often effective,
however, the treatments remain imperfect, and require improvement.
Further research is needed in all forms of vasculitis. Greater
knowledge of these diseases will lead to better treatments and, some
day, to cures.
Will my children or other family members get it?
Vasculitis is not contagious. Despite the fact that infections probably
play a role in many forms of vasculitis, one cannot acquire vasculitis
from contact with a vasculitis patient. In addition, despite the fact
that genes probably play a role in susceptibility to some forms of
vasculitis, it is unusual for vasculitis to occur in more than one
member of the same family. Thus, vasculitis is not a heritable
disorder. All of these points illustrate the fact that the causes of
vasculitis are complex. In all likelihood, patients develop vasculitis
because of the simultaneous occurrence of multiple risk factors, most
of which remain poorly understood.
Does diet affect vasculitis?
This is one of the most commonly-asked questions by patients with
vasculitis. All patients want to do whatever is within their power to
help treat their disease. Unfortunately, there is presently no evidence
that a person’s diet affects susceptibility to vasculitis, or that
consuming or avoiding certain foods or beverages affects the course
of the disease. In general, we advocate eating a balanced, “heart–
healthy” diet. Avoidance of excessive calories may be very important,
particularly in patients on steroids who are at risk for weight gain. We
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believe that some vitamins are appropriate, but are skeptical about
many “alternative” health remedies. Many of these are poorly-tested,
unregulated, and expensive. Some may even be dangerous.
Will my vasculitis return?
After patients achieve remission from their vasculitis, it is logical for
them to wonder if their disease will ever return. The answer, which is
often difficult to give with certainty, depends in large part on the
patient’s specific type of vasculitis. For example, some types of
vasculitis, such as Henoch-Schönlein purpura (HSP) or vasculitis
caused by a medication, tend to be self-limited and resolve on their
own. Other forms of vasculitis (e.g., Buerger’s disease, a disease
strongly associated with cigarette smoking) resolve with institution of
the definitive treatment: smoking cessation. Still other forms behave
less predictably, never coming back in some patients but
demonstrating a tendency to recur eventually in up to half or more of
all cases. Wegener’s granulomatosis, giant cell arteritis, Takayasu’s
arteritis, microscopic polyangiitis, and other types of vasculitis fall
into the category of diseases that often “flare” following the
achievement of remission. Sometimes flares occur when patients go
off of their treatments. Other times vasculitis flares when treatments
are lowered in attempts to avoid side-effects.
At the present time, the ability of doctors to predict who will suffer
disease flares and who will maintain in long-term remissions (or be
cured) needs refinement. Progress in this area will come through
research.
How should I guard against the occurrence of a disease flare?
We believe that several points are worth keeping in mind:
First, the symptoms of flares are usually very similar to the ones
patients had when their diseases began. If headaches signaled the
beginning of giant cell arteritis before, then the recurrence of
headaches may indicate a disease flare. If leg ulcers began as painful
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red lumps on the leg the first time, then the return of painful red
lumps may mean that vasculitis is back. Patients must become
experts about their own manifestations of vasculitis so that they
can recognize them immediately, consult their doctors, and begin
appropriate treatment before serious damage occurs.
Second, patients must learn to know their own bodies, and report to
their doctors any feelings or findings that are unusual. New
symptoms may not only indicate a vasculitis flare, they may also
herald complications of treatment such as infections.
Finally, because vasculitis treatments require careful monitoring by
doctors, patients should discuss any changes in treatment with their
physicians. Increasing or decreasing medications without consulting a
physician may lead to trouble.
Why do I have to have bloodwork checked frequently?
It’s simple (you’ve probably heard us say it before): “A watched kettle
doesn’t boil”. The more detailed answer is that in the management of
patients with vasculitis, we are concerned not only with treating the
disease, but also with preventing side effects of treatment. The lab
work that we assess regularly is designed to help us accomplish both
of these goals.
What types of tests do we check?
Regardless of the type of vasculitis and the exact type of medication
that a patient takes, similar types of tests must be monitored. These
tests are: 1) a complete blood count; 2) tests of kidney function
including a urinalysis; and 3) liver function tests. The table below
outlines the importance behind checking each of these tests.
Sometimes other tests are required, too, for special circumstances, as
when a patient is on a “blood thinner” such as coumadin.
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How often should my blood be checked?
This depends on the specific medicine or medicines that you take.
Patients on cyclophosphamide (Cytoxan) should have their counts
checked every 2 weeks. Patients on most other kinds of medications
used to treat vasculitis (Methotrexate, Azathioprine) usually only need
to have their blood work checked monthly. If some laboratory tests
are abnormal or nearly so, then more frequent monitoring may be
required.
Complications
Many cases of vasculitis are minor and either run their course without
treatment, such as some cases of Henoch-Schonlein purpura, or can
be effectively controlled by treatment.
However, some cases of vasculitis are severe and involve major organ
systems. In these cases, damage to the organs may occur before
treatment has time to work, or the condition may be so severe as to
resist treatment. These cases can result in major organ damage or
death.
Even when treatment for vasculitis is initially successful, the
condition may recur later and require further treatment. Giant cell
arteritis, Wegener's granulomatosis and Takayasu's arteritis all are
types of vasculitis that often recur after initial remission. In other
cases, vasculitis may never completely go away and requires ongoing
treatment.
Coping skills
When vasculitis is identified and treated early, the prognosis is usually
good. One of your greatest challenges may be coping with any side
effects of your medication. The following suggestions may help:
Understand your condition. Learn everything you can about
vasculitis and its treatment. Know the possible side effects of any
medications you take, and report any changes in your health to your
doctor.
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Eat a healthy diet. Eating well can help prevent potential problems
that can result from your medications, such as thinning bones, high
blood pressure and diabetes. Emphasize fresh fruits and vegetables,
whole grains, and lean meats and fish, while limiting salt, sugar and
alcohol.
Be sure to get adequate amounts of calcium and vitamin D. If you
find it hard to get calcium from your diet because you can't eat dairy
products, for example, try calcium supplements, which are often
combined with vitamin D. Supplements are effective, inexpensive,
and generally well tolerated and well absorbed if taken properly.
Sometimes calcium supplements can cause constipation. If this is a
problem for you, drink more water and try using a fiber supplement.
In addition, check the type of calcium you're using. Calcium
phosphate and calcium citrate tend to be less constipating than other
types.
Good food sources of calcium include milk; low-fat plain yogurt;
Swiss, cheddar and ricotta cheeses; broccoli; canned salmon with the
bones; and orange juice and other products, such as tofu, fortified
with calcium.
Exercise regularly. Regular aerobic exercise, such as walking, can
help prevent bone loss, high blood pressure and diabetes. It also
benefits your heart and lungs. In addition, many people find that
exercise improves their mood and overall sense of well-being. If
you're not used to exercising, start out slowly and build up gradually.
Your doctor can help you plan an exercise program that's right for
you.
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Outlook
The outlook for people who have vasculitis varies depending on both
the type and severity of the vasculitis. The course of a case of
vasculitis can be hard to predict. Treatment is often very effective if
vasculitis is diagnosed and treated early. Vasculitis may disappear or
go into a period of remission. In some cases, it may be a chronic
problem, requiring ongoing treatment, or it may come back (“flare”)
after a period of remission. In more severe cases, treatment may not
help, and vasculitis can lead to disability or even death.
Much is still unknown about vasculitis. There are many different
types of vasculitis, but overall it is a fairly rare condition in the
general population. Additional research is needed to learn more about
the various types of vasculitis and their causes, treatments, and
remission patterns.
Vasculitis
Also called: Angiitis, Arteritis
Vasculitis is an inflammation of the blood vessels. It happens when
the body's immune system attacks the blood vessel by mistake. The
cause is often unknown. Vasculitis can affect arteries, veins and
capillaries. Arteries are vessels that carry blood from the heart to the
body's organs. Veins are the vessels that carry blood back to the
heart. Capillaries are tiny blood vessels that connect the small arteries
and veins.
When a blood vessel becomes inflamed, it can Narrow, making it
more difficult for blood to get through close off completely so that
blood can't get through stretch and weaken so much that it bulges
and may burst and cause dangerous bleeding inside the body
Symptoms of vasculitis can vary, but usually include fever, swelling
and a general sense of feeling ill. The main goal of treatment is to
stop the inflammation. Steroids and other medicines to stop
inflammation are often helpful
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The list of signs and symptoms mentioned in various sources for
Vasculitis includes the 13 symptoms listed below:
Fever
Fatigue
Weight loss
Diffuse aches
Diffuse pains
Oligoarthritis
Neuropathy
Diminished pulses
Pulse discrepancy
Claudication
Hypertension
Visual blurring
Diplopia
Note that Vasculitis symptoms usually refers to various symptoms
known to a patient, but the phrase Vasculitis signs may refer to those
signs only noticable by a doctor.
More ways to research these symptoms: To research other
symptoms use the symptom center, or to research causes of more
than one symptom in combination, try multi-symptom search.
Diplopia - see all causes of Diplopia
Fatigue - see all causes of Fatigue
Fever - see all causes of Fever
Hypertension - see all causes of High blood pressure
Neuropathy - see all causes of Neuropathy
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Visual blurring - see all causes of Visual blurring
Weight loss - see all causes of Weight loss
Vasculitis as a cause of symptoms or medical conditions
When considering symptoms of Vasculitis, it is also important to
consider Vasculitis as a possible cause of other medical conditions.
The Disease Database lists the following medical conditions that
Vasculitis may cause:
Alopecia
Aneurysms
Aseptic necrosis of bone
Cerebellar syndrome
Cerebrovascular accident
Gastrointestinal bleeding
Glomerulonephritis
Haematuria
Hypertension, systemic
Livedo reticularis
Nail abnormality
Peripheral neuropathy
Purpura
Retinal neovascularization
Scleritis
Urticaria
Vesiculo-bullous rash
About signs and symptoms of Vasculitis:
The symptom information given below attempts to provide a list of
some possible signs and symptoms of Vasculitis, which may not be
fully accurate, and may not be the full list of Vasculitis signs or
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Vasculitis symptoms. Furthermore, signs and symptoms of Vasculitis
may vary on an individual basis for each patient.
Symptoms
• 1 Classification
o 1.1 Large vessel vasculitis
o 1.2 Medium vessel vasculitis
o 1.3 Small vessel vasculitis
2 Symptoms and signs
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3 Diagnosis
4 Treatment
5 References
6 External links
Classification
Vasculitides can be classified by the size of the blood vessel that they
predominantly affect.
Large vessel vasculitis
Takayasu arteritis. Primarily affects the aorta and its main branches.
At least 3 out of 6 criteria yields sensitivity and specificity of 90.5 and
97.8%:
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onset < 40 years
claudication of extremities
decreased pulsation of one or both brachial arteries
at least 10 mmHg systolic difference in both arms
bruit over one or both carotid arteries or abdominal aorta
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arteriographic narrowing of aorta, its primary branches, or large
arteries in upper or lower extremities
Giant cell (temporal) arteritis. Chronic vasculitis of both large and
medium vessels, primarily affecting cranial branches of the arteries
arising from the aortic arch. At least 3 out of 5 criteria yields
sensitivity and specificity of 95 and 91%:
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Age at onset ≥ 50 years
New onset headache with localized tenderness
Temporal artery tenderness or decreased pulsation
Elevated ESR ≥ 50 mm/hour Westergren
Temporal artery biopsy showing vasculitis with mononuclear
cell infiltrate or granulomatous inflammation, usually with
multinucleated giant cells
Medium vessel vasculitis
Polyarteritis nodosa. Systemic necrotizing vasculitis and
aneurysm formation affecting both medium and small arteries.
If only small vessels are affected, it is called microscopic
polyangiitis, although it is more associated with Wegener's
granulomatosis than to classic PAN. At least 3 out of 10 criteria
yields sensitivity and specificity of 82 and 87%:
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unexplained weight loss > 4 kg
livedo reticularis
testicular pain
myalgias, weakness
mononeuropathy or polyneuropathy
new onset diastolic blood pressure > 90 mmHg
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elevated serum BUN (> 40 mg/dL) or serum creatinine
(> 1.5 mg/dL)
hepatitis B infection
arteriographic abnormalities
arterial biopsy showing polymorphonuclear cells
Wegener's granulomatosis. Systemic vasculitis of medium and
small arteries, including venules and arterioles. Produces
granulomatous inflammation of the respiratory tracts and
necrotizing, pauci-immune glomerulonephritis. Most common
cause of saddle nose deformity in USA (nose flattened due to
destruction of nasal septum by granulomatous inflammation).
Almost all patients with WG has c-ANCA, but not vice versa.
Current treatment of choice is cyclophosphamide. At least 2 out
of 4 criteria yields sensitivity and specificity of 88 and 92%.
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nasal or oral inflammation (oral ulcers or purulent/bloody
nasal discharge, may be painful)
abnormal CXR showing nodules, infiltrates, cavities
microscopic hematuria or RBC casts
vessel biopsy shows granulomatous inflammation
Kawasaki disease. Usually in children, it affects large, medium,
and small vessels, prominently the coronary arteries. Associated
with a mucocutaneous lymph node syndrome. Diagnosis
requires fever lasting five days or more with at least 4 out of 5
criteria:
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bilateral conjunctival injection
injected or fissured lips, injected pharynx, or strawberry
tongue
erythema of palms/soles, edema of hands/feet,
periungual desquamation
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polymorphous rash
cervical lymphadenopathy (at least one node > 1.5 cm)
Isolated CNS vasculitis. Affects medium and small arteries over
a diffuse CNS area, without symptomatic extracranial vessel
involvement. Patients have CNS symptoms as well as cerebral
vasculitis by angiography and leptomeningeal biopsy
Small vessel vasculitis
There are several vasculitides that affect small vessels.[2]
Churg-Strauss arteritis. Affects medium and small vessels with
vascular and extravascular granulomatosis. Classically involves
arteries of lungs and skin, but may be generalized. At least 4
criteria yields sensitivity and specificity of 85 and 99.7%.
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asthma (history of wheezeing or presently wheezing)
eosinophilia > 10% on CBC
mononeuropathy or polyneuropathy
migratory or transient pulmonary opacities on CXR
paranasal sinus abnormalities
vessel biopsy showing eosinophils in extravascular areas
Microscopic polyarteritis/polyangiitis. Affects capillaries,
venules, or arterioles. Thought to be part of a group that
includes Wegeners since both are associated with ANCA and
similar extrapulmonary manifestations. Patients do not usually
have symptomatic or histologic respiratory involvement.
Hypersensitivity vasculitis. Usually due to a hypersensitivity
reaction to a known drug. There is presence of skin vaculitis
with palpable petechiae or purpura. Biopsy of these lesions
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reveal inflammation of the small vessels, termed
leukocytoclastic vasculitis, which is most prominent in
postcapillary venules. At least 3 out of 5 criteria yields
sensitivity and specificity of 71 and 84%:
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age > 16
use of possible triggering drug in relation to symptoms
palpable purpura
maculopapular rash
skin biopsy showing neutrophils around vessel
Henoch-Schonlein purpura. Systemic vasculitis due to tissue
deposition of IgA-containing immune complexes. Biopsy of
lesions shows inflammation of small vessels. It is considered a
form of hypersensitivity vasculitis but is distinguished by
prominent deposits of IgA. This is the most common vasculitis
in children. Presence of 3 or more criteria yielded sensitivity of
87% while less than 2 criteria yielded hypersensitivity vasculitis
in 74%
palpable purpura (usually of buttocks & legs)
• bowel angina
• GI bleed
• hematuria
• onset < 20 years
no new medications
Essential cryoglobulinemic vasculitis. Most often due to
hepatitic C infection, immune complexes of cryoglobulins --proteins that consists of immunoglobulins and complement
and precipitate in the cold while dissolving upon rewarming --are deposited in walls of capillaries, venules, or arterioles.
Therefore, complement will be low with histology showing
vessel inflammation with immune deposits.
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Vasculitis secondary to connective tissue disorders. Usually
secondary to systemic lupus erythematosus (SLE), rheumatoid
arthritis (RA), relapsing polychondritis, Behçet's disease, and
other connective tissue disorders.
Vasculitis secondary to viral infection. Usually due to hepatitis
B and C, HIV, cytomegalovirus, Epstein-Barr virus, and Parvo
B19 virus.
Symptoms and signs
Patients usually present with systemic symptoms with single or
multiorgan dysfunction. Common (and nonspecific) complaints
include fatigue, weakness, fever, arthralgias, abdominal pain,
hypertension, renal insufficiency, and neurologic dysfunction.
The following symptoms should raise a strong suspicion of a
vasculitis.
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Mononeuritis multiplex. Also known as asymmetric
polyneuropathy is highly suggestive of vasculitis, since
diabetes is the only other cause of this.
Palpable purpura. If patients have this in isolation, it is
most likely due to cutaneous leukocytoclastic vasculitis. If
the purpura is in combination with systemic organ
involvement, it is most likely to be Henoch-Schonlein
purpura or microscopic polyarteritis.
Pulmonary-renal syndrome. Patients with hemoptysis and
renal involvement are likely to have Wegener's
granulomatosis, microscopic polyangiitis, or anti-GBM
disease (Goodpasture's syndrome).
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Diagnosis
A detailed history is important to elicit any recent
medications, any risk of hepatitis infection, or any recent
diagnosis with a connective tissue disorder such as
systemic lupus erythematosus (SLE). A thorough physical
exam is needed as usual.
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Lab tests. Basic lab tests may include a CBC, chem-7
(look for creatinine), muscle enzyme, liver function tests,
ESR, hepatitis seroloties, urinalysis, CXR, and EKG.
Additional, more specific tests include:
o Antinuclear antibody (ANA) test can detect an
underlying connective tissue disorder, especially
SLE
o Complement levels that are low can suggest mixed
cryoglobulinemia, hepatitis C infection, and SLE,
but not most other vasculitides.
Antineutrophil cytoplasmic antibody (ANCA) may highly
suggest Wegener's granulomatosis, microscopic
polyangiitis, Churg-Strauss syndrome, or drug-induced
vasculitis, but is not diagnostic.
Electromyography. It is useful if a systemic vasculitis is
suspected and neuromuscular symptoms are present.
Arteriography. Arteriograms are helpful in vasculitis
affecting the large and medium vessels but not helpful in
small vessel vasculitis. Angiograms of mesenteri or renal
arteries in polyarteritis nodosa may show aneurysms,
occlusions, and vascular wall abnormalities. Arteriography
are not diagnostic in itself if other accessible areas for
biopsy are present. However, in Takayasu's arteritis,
where the aorta may be involved, it is unlikely a biopsy
will be successful and angiography can be diagnostic.
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Tissue biopsy. This is the gold standard of diagnosis
when biopsy is taken from the most involved area.
Treatment
Treatment is targeted to the underlying cause. However,
most vasculitis in general are treated with steroids (e.g.
methylprednisolone) because the underlying cause of the
vasculitis is due to hyperactive immunological damage.
Immunosuppressants such as cyclophosphamide and
azathioprine may also be given.
A systematic review of antineutrophil cytoplasmic
antibody (ANCA) positive vasculitis identified best
treatments depending on whether the goal is to induce
remission or maintenance and depending on severity of
the vasculitis.
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