Download Apoplexy, CVA, TIA

Vasculities, Polymyalgia
Rheumatica, Giant Cell Arteritis
(Temporal Arteritis)
Victor Politi, M.D. FACP
Medical Director, SVCMC
Physician Assistant Program
Vasculitis
• “Vasculitis” is a general term for a group of
diseases that involve inflammation in blood
vessels.
• Blood vessels of all sizes may be affected,
from the largest vessel in the body (the
aorta) to the smallest blood vessels in the
skin (capillaries).
• The size of blood vessel affected varies
according to the specific type of vasculitis.
Overview
• The effects of vasculitis that result
from damage to the blood vessel
include decreased function due to
decreased blood flow (ischemia),
death of some or all of an organ due
to absent blood flow (infarction), or
bleeding into the skin or other part
of the body due to rupture of the
blood vessel wall.
WHAT CAUSES VASCULITIS?
• Sometimes, it is precipitated by a
reaction to a drug or other substance.
(This is known as hypersensitivity
vasculitis.)
WHAT CAUSES VASCULITIS?
• In still other cases, it occurs in
conjunction with a viral illness, such
as hepatitis B or C, HIV,
cytomegalovirus, Epstein-Barr virus,
and Parvo B19 virus.
• Some common symptoms include:
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Fatigue
Weakness
Fever
Joint pains
Abdominal pain
Kidney problems (bloody urine, dark urine)
Nerve problems (numbness, weakness, pain)
• Tissue biopsy is a critical component of the
diagnostic process.
• The tissue sample is taken from an area
thought to be involved in the vasculitis.
• In vasculitis affecting the larger vessels,
an arteriogram may be useful. This test
involves injecting dye into the arteries,
which makes them visible on x-ray.
TYPES OF VASCULITIS
• There are many different types of
vasculitis.
– They are classified according to the
type and location of the blood vessels
that are generally involved.
Large vessel vasculitis
• Large vessel vasculitis — The types
of vasculitis that affect large
arteries include Takayasu arteritis
and giant cell (temporal) arteritis.
Large vessel vasculitis
• Involvement of the main artery of
the body, the aorta, can sometimes
occur in association with other
illnesses such as ankylosing
spondylitis, rheumatoid arthritis, and
relapsing polychondritis.
Large vessel vasculitis
Takayasu arteritis
• Takayasu arteritis primarily affects
the main artery that receives blood
from the heart (aorta) and its
branches.
• The inflammation may be localized to
a portion of the aorta in the chest or
abdomen and branches.
Large vessel vasculitis
Takayasu arteritis
• The involvement of large arteries may
lead to symptoms such as pain and
weakness with use of the arms or legs
(claudication).
• Other organs such as the intestines
(abdominal pain after eating), heart
(chest pain with exertion), or brain.
On the right is an example of an abnormal aortic arch in a patient with Takayasu's,
with obvious dilation of the ascending aorta on the left side of the picture
Large vessel vasculitis
Giant cell arteritis
• Giant cell arteritis may also affect
the aorta and its branches.
• Frequent involvement of the arteries
of the face and scalp, particularly
those near the temples, accounts for
the other common name for this
disorder, temporal arteritis.
Large vessel vasculitis
Giant cell arteritis
• Giant cell arteritis is a disease that
nearly always affects people older
than 50 years of age.
• Among a million people 50 or older,
approximately 2000 may be affected
at any one time.
Large vessel vasculitis
Giant cell arteritis
• In addition to general symptoms
associated with inflammation,
headache, tiring of jaw muscles
during chewing, and visual changes or
loss of vision are suggestive of this
disease.
Large vessel vasculitis
Giant cell arteritis
• The diagnosis is suspected based upon
symptoms, the finding of an elevated
level of a blood test (erythrocyte
sedimentation rate or C-reactive
protein), and a confirmatory biopsy of
an artery (usually one or both
temporal arteries).
Medium sized vessel vasculitis
• Some types of vasculitis appear to
spare the aorta and affect medium
sized arteries instead.
• Polyarteritis is the term used for this
disorder when it occurs in the
absence of any other disease.
Medium sized vessel vasculitis
Polyarteritis nodosa (PAN)
• Polyarteritis nodosa is a term that
refers to inflammation of medium to
small arteries.
• In the skin the inflammation results
in thickened nodular (nodose) vessels
that can be felt or sometimes seen.
Medium sized vessel vasculitis
Polyarteritis nodosa
• Damage to the nerves of the arms or
legs, to the kidneys, the intestines,
and the heart may occur.
• The diagnosis is suspected when
several organs of the body are being
damaged at the same time.
Medium sized vessel vasculitis
Polyarteritis nodosa
• Skin abnormalities are very common
in PAN and may include purpura,
livedo reticularis, ulcers, nodules or
gangrene.
• Skin involvement occurs most often
on the legs and is very painful.
Medium sized vessel vasculitis
Polyarteritis nodosa
• Testing for the presence of
antineutrophil cytoplasmic antibodies
(ANCA) in the blood is helpful
because these are frequently present
in patients with polyarteritis or
polyangiitis.
Medium sized vessel vasculitis
Polyarteritis nodosa
• Treatment of PAN has improved
dramatically in the past couple of decades.
• Before the availability of effective
therapy, untreated PAN was usually fatal
within weeks to months.
• Most deaths occurred as a result of kidney
failure, heart or gastrointestinal
complications.
Medium sized vessel vasculitis
Polyarteritis nodosa
• However, effective treatment is now
available for PAN. After diagnosis,
patients are treated with high doses of
corticosteroids.
• Other immunosuppressive drugs are also
added for patients who are especially ill. In
most cases of PAN now, if diagnosed early
enough the disease can be controlled, and
often cured.
Medium sized vessel vasculitis
• Other diseases that can affect the
medium sized arteries include
Kawasaki disease and isolated central
nervous system vasculitis
Small vessel vasculitis
• Several different types of vasculitis
can affect small vessels such as
arterioles, capillaries, and small veins
(venules).
• These disorders may appear very
similar based upon biopsy results, but
are distinguished from one another
by other features.
Small vessel vasculitis
Churg-Strauss Vasculitis
• Churg-Strauss vasculitis occurs
almost exclusively in people who have
asthma.
• It is likely to cause lung damage.
• ANCA testing is valuable.
• Biopsy is useful to confirm the
diagnosis.
Small vessel vasculitis
Wegener's granulomatosis
• Wegener's granulomatosis
characteristically affects the nose and
sinuses, the lungs, and the kidneys.
• Almost all those with Wegener's
granulomatosis have a positive ANCA blood
test.
• Biopsy of the lining of the nose, a sinus,
part of a lung, or kidney may confirm the
diagnosis.
Small vessel vasculitis
Henoch-Schönlein purpura
• Henoch-Schönlein purpura most often
affects children but can occasionally cause
disease in adults.
• Hallmarks of this illness are abdominal and
joint pain, a skin rash consisting of small,
red to purple, slightly raised areas, and
kidney involvement that causes the urine to
appear bloody or darkly colored, like tea or
coffee.
Small vessel vasculitis
Henoch-Schönlein purpura
• The diagnosis of Henoch-Schönlein purpura
is suggested by the symptoms and
characteristic skin rash.
• Skin or kidney biopsy can confirm the
diagnosis, especially if there are increased
amounts of a specific class of antibody
proteins (immunoglobulin A or IgA) in
affected blood vessels or within the
kidney.
Small vessel vasculitisCryoglobulinemia
• Cryoglobulins are complexes of the body's
infection fighting proteins (antibodies,
immunoglobulins) with the proteins that are
their targets (antigens).
• When the serum of the blood of patients
with cryoglobulinemia is cooled, the
complexes become so large that they form
visible clumps (precipitates, cryoglobulins).
Small vessel vasculitisCryoglobulinemia
• Among people with cryoglobulinemic
vasculitis, many have chronic
infections.
• The most common is caused by the
hepatitis C virus.
Small vessel vasculitisCryoglobulinemia
• Two features of this type of
vasculitis are the appearance of crops
of raised red bumps on the legs and
inflammation of the kidneys
(glomerulonephritis).
Small vessel vasculitisCryoglobulinemia
• A blood test for cryoglobulins and a
characteristic appearance of a skin or
kidney biopsy specimen confirms the
diagnosis.
• the hand from the same patient at
different times. The image on the
left is normal and the one on the
right shows the patient in the midst
of a flare of cryoglobuinemic
vasculitis.
Small Vessel VasculitisHypersensitivity vasculitis
• Inflammation of small blood vessels,
that cannot be classified as any of
the previous disorders, and which
occurs after someone has been
exposed to a medication that could
cause an allergic (hypersensitivity)
reaction may lead to a diagnosis of
hypersensitivity vasculitis.
Small vessel vasculitis
• Small vessel vasculitis may also be
seen in some patients with
rheumatoid arthritis, systemic lupus
erythematosus, inflammatory muscle
diseases (polymyositis and
dermatomyositis), Sjögren's
syndrome.
HOW IS VASCULITIS
TREATED?
• In hypersensitivity vasculitis, removal
of the offending substance is often
enough.
• Some patients may need a short
course of steroid therapy. Others
benefit from nonsteroidal
antiinflammatory drugs such as
ibuprofen.
HOW IS VASCULITIS
TREATED?
• The exact treatment of the other
types of vasculitis will be dependent
on the specific type of vasculitis and
the areas/organs that are involved.
HOW IS VASCULITIS
TREATED?
• Some measures that may be
necessary include:
– Use of steroids, such as prednisone.
Steroids may be taken orally in some
cases or high doses may be needed and
given intravenously.
HOW IS VASCULITIS
TREATED?
• For more serious types of vasculitis,
or when steroids cannot be tapered
because of recurrent vasculitis, other
"cytotoxic" medications are used.
– These medicines suppress the immune
system and interfere with the function
of cells that participate in the vasculitic
process.
HOW IS VASCULITIS
TREATED?
– The use of one such drug,
cyclophosphamide, has dramatically
improved the outlook for patients with
some types of vasculitis.
Behcet’s disease
• is most common along the “Old Silk
Route”, which spans the region from
Japan and China in the Far East to
the Mediterranean Sea, including
countries such as Turkey and Iran.
Behcet’s disease
• In Japan, Behcet’s disease ranks as a
leading cause of blindness.
Behcet’s disease
• Because of the diversity of blood
vessels it affects, manifestations of
Behcet’s may occur at many sites
throughout the body.
Behcet’s disease
• Behcet’s is one of the few forms of
vasculitis in which there is a known
genetic predisposition.
• The presence of the gene HLA–B51 is
a risk factor for this disease- many
people possess the gene, but
relatively few develop Behcet’s.
Behcet’s disease
• Diagnosis is based on the occurrence of
symptoms and signs that are compatible
with the disease, the presence of certain
features that are particularly
characteristic (e.g., oral or genital
ulcerations), elimination of other possible
causes of the patient’s presentation, and —
whenever possible — proof of vasculitis by
biopsy of an involved organ.
Buerger’s Disease
• characteristic pathologic findings —
acute inflammation and thrombosis
(clotting) of arteries and veins —
affecting the hands and feet.
• Another name for Buerger’s Disease
is thromboangiitis obliterans.
Buerger’s Disease
• The classic Buerger’s Disease patient
is a young male (e.g., 20–40 years old)
who is a heavy cigarette smoker.
• More recently, however, a higher
percentage of women and people over
the age of 50 have been recognized
to have this disease.
Buerger’s Disease
• Initial symptoms often include
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Claudication
numbness and/or tingling in the limbs
Raynaud’s phenomenon
Skin ulcerations and gangrene of the
digits
Buerger’s Disease
• The association of Buerger’s Disease
with tobacco use, particularly
cigarette smoking, cannot be
overemphasized.
• Most patients with Buerger’s are
heavy smokers, but some cases occur
in patients who smoke “moderately”
Buerger’s Disease
• Certain angiographic findings are
diagnostic of Buerger’s.
• These findings include a “corkscrew”
appearance of arteries that result from
vascular damage, particularly the arteries
in the region of the wrists and ankles.
• Angiograms may also show occlusions
(blockages) or stenoses (narrowings) in
multiple areas of both the arms and legs.
Buerger’s Disease
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On the right, is an abnormal angiogram of an arm demonstrating the classic
“corkscrew” appearance of arteries to the hand. The changes are
particularly apparent in the blood vessels in the lower right hand portion of
the picture (the ulnar artery distribution).
Buerger’s Disease
• It is essential that patients with
Buerger’s disease stop smoking
immediately and completely.
• This is the only treatment known to
be effective in Buerger’s disease.
• Patients who continue to smoke are
generally the ones who require
amputation of fingers and toes.
Polymyalgia Rheumatica (PMR)
• An inflammatory disorder that causes
widespread muscle aching and
stiffness, especially in the neck,
shoulders, thighs and hips.
Overview
• Just what triggers PMR isn't known,
but the cause may be a problem with
the immune system, perhaps involving
both genetic and environmental
factors.
• Aging also appears to play a role —
the disease almost always occurs in
people age 50 and older.
Overview
• PMR usually goes away on its own in a
year or two — often as mysteriously
as it came.
• Mild symptoms - NSAIDs
• Severe pain - corticosteroids
(prednisone)
• In PMR, the aching is located primarily
around the shoulders and hips
Causes
• In PMR, inflammation occurs when
white blood cells attack the lining of
the joints (synovium).
• Researchers aren't sure what causes
this abnormal immune system
response, but they suspect that as
with many disorders, both genetic and
environmental factors are involved.
Points to Remember!
• Aching and stiffness come on quickly in
PMR but there are often no visible signs of
swelling and inflammation on examination,
making it difficult to diagnose.
• Symptoms are worst at night and on rising
in the morning.
• Symptoms respond briskly to low doses of
corticosteroids, but some symptoms may
recur as the dose is lowered.
Temporal Arteritis
• A systemic panarteritis affecting
medium sized and large vessels in
patients over the age of 50
• This condition is also referred to as
Giant Cell Arteritis
Giant Cell Arteritis
• Referred to as temporal arteritis
since that artery is frequently
involved
• Other extracranial branches of the
carotid artery are also usually
involved
• Approximately 50% of patients also
have polymyalgia rheumatica
Classic Symptoms
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Headache
scalp tenderness
visual symptoms
jaw claudication
throat pain
Classic Symptoms
• The temporal artery is usually normal
on exam but may be nodular, enlarged,
tender, or pulseless
• Occlusive arteritis of the posterior
ciliary branch of the ophthalmic
artery results in blindness
• In an older patient with FUO, an
elevated ESR, and normal WBC count,
giant cell arteritis must be
considered even in the absence of
well-known symptoms of headache and
jaw claudication
Lab Findings
• In 90% of cases - elevated ESR
• Elevated C-reactive protein
• Mild normochromic, normocytic
anemia and thrombocytosis
• Elevated Alk Phos
Treatment
• Goal - prevention of blindness - once
blindness develops it is usually
permanent
• Therapy with prednisone, 60mg daily
for one-two months before tapering
• Temporal artery biopsy - (positive
findings may be present up to two
weeks after starting prednisone)
Treatment
• When only symptoms of polymyalgia
rheumatica present - temporal artery
biopsy not necessary
• In adjusting prednisone dosage- ESR
useful but not absolute reference
• Treat the patient not the ESR!
PMR
• Polymyalgia rheumatica
• age over 50
• Stiffness and pain to shoulder and hip
muscles
• inflammatory polyarthritis/joint
effusions to knees and other joints
• normal CPK and elevated ESR
PMR
• DX--muscle biopsy shows atrophy
without necrosis or inflammation
• RX-- nsaids/ steroids
• Diff Dx include
dermatomyositis/polymyositis
Follow-up
• Thoracic aortic aneurysms occur 17
times more frequently in giant cell
arteritis cases • This can happen at any time but
typically occurs approximately seven
years after the diagnosis
Questions ???