Transcript 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
• There are many different types of
blood vessels in the body.
• Any particular vessel is part of a
large vascular "tree" that includes
large and medium sized arteries and
smaller and smallest arterial branches
(arterioles).
Overview
• These branches eventually reach all
the tissues of the body, delivering
oxygen and nutrients to a network of
tiny vessels, called capillaries, that
also remove wastes.
Overview
• The capillaries drain into the venous
system.
• The smallest veins are venules; these
connect like the tributaries of a river
to form larger and larger veins.
Overview
• Vasculitis can affect any of these
different types of blood vessels.
Inflammation can affect the lining of
the vessels (endothelium) or the wall
of an artery or vein. The damaged
vessel does not function normally, and
tissues that the vessel normally
serves may then be affected.
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.
Overview
• Because vasculitis is a process that
involves inflammation, it is usually
accompanied by other features such
as fever, or symptoms of involuntary
weight loss and fatigue.
Overview
• Fortunately, available treatments for
vasculitis are helpful, especially in the
acute phase.
• Long term therapy presents
challenges because of side effects of
the medications typically used to
treat the disorder.
WHAT CAUSES VASCULITIS?
• In most cases, the cause is unknown.
• It is likely that a combination of
factors causes the inflammatory
process to be set in motion.
WHAT CAUSES VASCULITIS?
• Vasculitis can occur in conjunction
with another illness, such as lupus
erythematosus or rheumatoid
arthritis.
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.
WHAT ARE THE
SYMPTOMS?
• Symptoms vary from patient to
patient, and are dependent in part on
the type of vasculitis.
• 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)
• The diagnosis of vasculitis is often
difficult, as the patient's symptoms
may suggest many other illnesses.
• Begin with a careful history and
physical exam
– You may be able to detect signs of organ
problems suggestive of a vasculitic
process.
• Laboratory tests can help pinpoint
the areas of the body affected.
• Tests may include those that examine
muscle, liver, or kidney function.
• Other common tests that may provide
useful information are additional
blood tests, urinalysis, chest x-ray,
and electrocardiogram.
• Tests of lung function may be needed
in some cases.
• Patients with evidence of nerve or
muscle involvement may undergo
nerve conduction studies and an
electromyogram (a test of muscle
function).
• 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.
• The diagnostic tests that are used
vary widely depending on the type of
vasculitis that is suspected.
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
• Only 1-3 cases are diagnosed in a year
among 1 million people in North America.
• The disease most often affects women
during the years from age 10 to about 40.
There is a 9:1 female predominance in this
disease.
• Although the disease has a worldwide
distribution, it appears to occur more
often in Asian women.
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
Takayasu arteritis
• The narrowing and irregularities that occur
at several sites, and the “corkscrew”
configuration of one vessel segment near
the junction of the two arteries.
• These changes, caused by inflammation in
the blood vessel wall, sometimes cause
complete blockage of the artery
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
• But similar involvement of blood
vessels can occur when vasculitis
develops in some people with
rheumatoid arthritis, systemic lupus
erythematosus, scleroderma
(systemic sclerosis), hairy cell
leukemia, and infectious forms of
hepatitis (hepatitis B and C).
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 (PAN)
• Polyarteritis nodosa is sometimes
termed “systemic necrotizing
vasculitis”, but this term is nonspecific as other forms of vasculitis
also have systemic and necrotizing
features.
Medium sized vessel vasculitis
Polyarteritis nodosa
• General symptoms due to
inflammation, and specific symptoms
due to bleeding, decreased blood flow
(ischemia), or irreversible damage to
organs due to the absence of blood
flow (infarction), suggest the
presence of polyarteritis.
Medium sized vessel vasculitis
Polyarteritis nodosa
• Most cases of PAN occur in the 4th or 5th
decade, although it can occur at any age.
• Men are twice as likely to be affected than
women.
• A minority of patients with PAN have an
active hepatitis B infection.
• In the rest of the cases, the cause(s) is
presently unknown, and the disease is said
to be “idiopathic” in nature.
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
• Peripheral neuropathies are very common
(50 to 70%). This includes tingling,
numbness and/or pain in the hands, arms,
feet, and legs.
• Central nervous system (CNS) lesions may
occur 2 to 3 years after the onset of PAN
and may lead to cognitive dysfunction,
decreased alertness, seizures and
neurologic deficits.
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
• Arteriography or biopsy of an
involved blood vessel is often
necessary to confirm the diagnosis.
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
• Although the disease is rare in the
United States, sporadic cases do
occur in patients who would not
appear to be at risk because of their
ethnic backgrounds (e.g., in
Caucasians or African–Americans).
Behcet’s disease
• In Japan, Behcet’s disease ranks as a
leading cause of blindness.
Behcet’s disease
• Behcet’s disease is virtually
unparalleled among the vasculitides in
its ability to involve blood vessels of
nearly all sizes and types, ranging
from small arteries to large ones, and
involving veins as well as arteries.
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
• Buerger’s disease is most common in
the Orient, Southeast Asia, India and
the Middle East, but appears to be
rare among African–Americans.
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
• Buerger’s disease can be mimicked by
a wide variety of other diseases that
cause diminished blood flow to the
extremities.
Buerger’s Disease
• Diseases with which Buerger’s
Disease may be confused include
atherosclerosis,endocarditis, other
types of vasculitis, severe Raynaud’s
phenomenon associated with
connective tissue disorders (e.g.,
lupus or scleroderma), clotting
disorders of the blood, and others.
Buerger’s Disease
• Angiograms of the upper and lower
extremities can be helpful in making
the diagnosis of Buerger’s disease.
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
•
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
• Although some people develop these
symptoms gradually, PMR can literally
appear overnight.
• People with the condition may go to
bed feeling fine, only to awaken in
pain the next morning.
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)
Signs/Symptoms
• PMR causes moderate to severe aching and
stiffness in the muscles in the hips, thighs,
shoulders, upper arms and neck.
• Most patients have pain in at least two of
these areas.
• Initially, pain may be on just one side of
the body, but as the disease progresses,
symptoms are likely to occur on both sides.
Signs/Symptoms
• Stiffness is usually worse in the
morning or after sitting or lying down
for long periods and may last up to an
hour.
Signs/Symptoms
• The aching and stiffness of PMR
often occur suddenly, but sometimes
may develop gradually. PMR can cause
other signs and symptoms including:
– Fatigue
– Unintentional weight loss
– Weakness or a general feeling of being
unwell
– Sometimes, a slight fever
• In the United States, approximately
15 percent of people with PMR have a
related condition called giant cell
arteritis, which causes the arteries in
the temples and sometimes in the
neck and arms to become swollen and
inflamed.
• In PMR, the aching is located primarily
around the shoulders and hips
Causes
• PMR is an arthritic syndrome that
causes the muscles to feel achy and
stiff due to mild inflammation of the
joints and surrounding tissues.
• Most of the inflammation occurs in
the hip and shoulder joints, but it
may develop elsewhere in the body as
well.
Causes
• In general, the inflammation isn't as
severe as that in inflammatory types
of arthritis, such as rheumatoid
arthritis.
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.
Causes
• Some evidence suggests a link
between PMR and certain viruses,
such as adenovirus, which causes
respiratory infections ranging from
the common cold to pneumonia; human
parvovirus B19, the source of an
infection that primarily affects
children; and human parainfluenza
virus.
Polymyalgia rheumatica
Screening and diagnosis
• The signs and symptoms of PMR are
similar to those of a number of other
conditions, including rheumatoid
arthritis and polymyositis — a disease
that causes muscle inflammation and
weakness.
Polymyalgia rheumatica
Screening and diagnosis
• Take a complete medical history
• Thorough physical exam
• Lab Studies
– CBC, Sed Rate, RF, C-reactive protein
Polymyalgia rheumatica
Complications
• The most serious complication of PMR
is giant cell arteritis.
– If left untreated, giant cell arteritis
may lead to vision loss, a stroke or an
aortic aneurysm
Polymyalgia rheumatica
Complications
– The exact relationship between
Polymyalgia Rheumatica and Giant Cell
Arteritis isn't clear,
• but about 15 % of people with PMR also
develop giant cell arteritis
• nearly 50% of those with giant cell arteritis
have PMR.
Polymyalgia rheumatica
Complications
• PMR itself causes few other serious
problems, but the corticosteroid
drugs used to treat the disease can
cause a number of complications.
– the risk of developing diabetes or bone
fractures is 2-5 times higher than the
risk for people not taking steroids.
Polymyalgia rheumatica
Treatment
• If the diagnosis of PMR is strongly
suspected, a trial of low dose
corticosteroids is given, usually in the form
of 10-15 mg of prednisone (Deltasone,
Orasone, etc.) per day.
• If PMR is present, these medications will
quickly control the pain.
• The response to corticosteroids can be
dramatic – sometimes, patients experience
improvement after only one dose – but the
symptoms may improve more slowly.
Polymyalgia rheumatica
Treatment
• However, if symptoms have not been
completely relieved after 2 to 3
weeks of treatment, the diagnosis of
PMR must be called into question and
other diagnoses should be considered.
Polymyalgia rheumatica
Treatment
• Unfortunately, nonsteroidal antiinflammatory drugs, such as
ibuprofen (Advil, Motrin, etc.) and
naproxen (Naprosyn), are ineffective
in the initial treatment of PMR.
Polymyalgia rheumatica
Treatment
• When symptoms have been controlled, the
dose of corticosteroid medication is
gradually decreased.
• The goal is to find the lowest dose that
keeps the individual patient comfortable.
• Some people can stop corticosteroids
within a year, but most will need a small
amount of this medication for 2 to 3 years,
sometimes longer, to keep aching and
stiffness under control.
• .
Polymyalgia rheumatica
Treatment
• Because the symptoms of PMR are so
sensitive to even small changes in
corticosteroid dose, it is not unusual
for some of the symptoms to return
as this medication is decreased.
• So both the blood tests and the
corticosteroid dose must be closely
monitored
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
•
•
•
•
•
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
Classic Symptoms
• Ischemic optic neuropathy of giant
cell arteritis may produce no
funduscopic findings for the first 2448 hours after the onset of blindness
Classic Symptoms
• In patients in whom giant cell
arteritis has affected the aorta or
its major branches
– asymmetry of pulses in the arms
– a murmur of aortic regurgitation
– bruits head near the clavicle resulting
from subclavian artery stenoses
Classic Symptoms
• In approximately 15% of patients large vessel involvement, particularly
thoracic aortic aneurysms, may occur
years after the diagnosis
• Diff Dx Takayasu’s Arteritis
Non-classic Symptoms
• 40% of patients present with nonclassic symptoms
– respiratory tract problem
• (dry cough)
– mononeuritis multiplex
• (painful paralysis of shoulder)
Non-Classic symptoms
• Fever of unknown origin – Giant cell arteritis accounts for 15% of
all cases of FUO in patients > 65
– frequently associated with rigors,sweats
– usually have normal WBC count (prior to
prednisone)
• 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 ???