Download Giant Cell Arteritis

Giant Cell Arteritis (GCA)
Mark Stradling, OMS IV
Introduction
Introduction
• Also know as temporal arteritis, cranial
arteritis, and granulomatous arteritis.
• Systemic inflammatory vasculitis of
unknown etiology.
• Affects medium- and large-sized arteries.
• Disease of the elderly population.
Introduction
• Results in systemic, neurologic, and
ophthalmologic complications.
• Visual loss is the most significant cause of
morbidity.
• Permanent visual impairment may occur in
up to 60% of patients with GCA.
• True neuro-ophthalmic emergency.
Pathophysiology
Pathophysiology
• Typically involves inflammation of the
aortic arch and its branches.
• Almost any artery, as well as veins, may be
affected occasionally.
• Likely determinant of arterial susceptibility
to GCA is the presence and/or quantity of
internal elastic lamina within the vessel
wall.
Pathophysiology
• Most commonly involved arteries:
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Extracranial vertebral arteries
Superficial temporal arteries
Posterior ciliary arteries
Opthalmic arteries
Pathophysiology
• Other commonly affected arteries:
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Internal carotid
External carotid
Central retinal arteries
Proximal aorta
Distal aorta
Subclavian arteries
Brachial arteries
Abdominal arteries
Histopathology
• Reveals inflammatory infiltrate surrounding
a fragmented internal elastic lamina within
the media of an arterial wall.
• Infiltrate consists predominantly of
mononuclear cells with giant cell formation.
• Mechanism is believed to be related to
dysfunction of cellular immunity, but
etiology is unknown.
Frequency
• Approximately 0.5-27 cases per 100,00
people aged 50 years or older.
• Annual incidence is higher in northern areas
of the US.
Mortality / Morbidity
Mortality / Morbidity
• In the milder form, patients may complain
of only generalized muscle aches and pains,
and unusual fatigue.
• Intermittent claudication occurs in about
50% of patients.
• In small percentage of patients, claudication
of the tongue or throat develops with eating
and repeated swallowing.
Mortality / Morbidity
• Visual symptoms are present in about 33%
of patients.
– 40-50% are transient
– 50-60% are permanent
• One of the more serious complication is the
onset of blindness from involvement of the
ophthalmic artery.
Mortality / Morbidity
• Permanent visual loss may be partial or
complete and may occur without warning
– 50% are unilateral
– 50% are bilateral
• Varied visual symptoms including blurring
of vision, diplopia, and loss of vision occur
in 36-60% of patients.
Mortality / Morbidity
• Involvement of major vessels (Aorta)
predisposes patients to higher risks for
death.
• In Evans and colleagues’ report, 50% of
those with thoracic aorta aneuryms died
suddenly from aortic dissection.
[Evans JM, Hunder GG: Polymyalgia and giant cell arteritis. Clin Geriatr Med 1998 Aug; 14(3): 455-73]
Mortality / Morbidity
• Race – incidence rates appear to be high in
Caucasians or European descent. This condition is
less commonin African Americans and Asians
• Sex: women are 2-4 times more likely to have
GCA than men.
• Age: mostly occurs in patients > age 50 – peaks at
the eighth decade; Rare in patients younger than
50 years of age.
Clinical Overview
General History
• Onset of GCA may be abrupt or insidious.
• Usually symptoms have been present for
weeks or months before the diagnosis is
established.
• Constitutional symptoms including
anorexia, fatigue, and weight loss are
present in most patients and may be an
initial finding.
Ophthalmic History
• 50% of patients with GCA eventually
experience visual symptoms (transient
visual blurring, diplopia, eye pain, sudden
loss of vision).
• Transient repeated episodes are usually
reversible.
• Sudden loss of vision is ominous and almost
always permanent.
Ophthalmic History
• The most common cause of vision loss is anterior
ischemic optic neuropathy (AION).
• Results from ischemia of the optic nerve head,
supplied mainly by the posterior ciliary arteries.
• Most AION is nonarteritic (87-91%).
• History of sudden painless loss of vision
frequently accompanies the patient with AION.
Systemic History
• May begin with symptoms of anorexia, fever,
malaise, myalgia, night sweats, and weight loss.
• Prodromal symptoms may occur for a few days or
may even stretch out to weeks.
• Hallmark symptom of GCA is its new-onset
localized headache.
• Localized to the temporal or occipital area, and
may be occasionally may be diffuse or bilateral.
Ophthalmic Exam
• Most common cause of vision loss is AION.
• Examination of the fundus may reveal optic disc
edema, with or without splinter hemorrhages
along the disc margin.
• Arteritic AION, as in GCA, typically presents with
a chalky white edematous optic disc.
• Automated visual field testing reveals an inferior
altitudinal defect, inferior nasal sectorial defect, or
central scotoma.
Ophthalmic Exam
• Other presentations include:
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Posterior ischemic optic neuropathy
Central retinal artery occlusion
Branch retinal artery occlusion
Choroidal ischemia
Ophthalmic Exam
• Neuro-ophthalmic manifestations include:
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Diplopia
Ptosis
Nystagmus
Internuclear ophthalmoplegia
Pupillary abnormalities
Systemic Exam
• Hallmark symptom is new-onset localized
headache
– Temporal area
– Occipital area
– Diffuse or bilateral
Systemic Exam
• Of interest is the scalp tenderness that
accompanies this headache – especially over the
temporal region
• Tenderness often induced by very gentle pressure.
• Hypersensitivity or hyperesthesia, ie when gently
stroking the patient’s hair, results in a
characteristic complaint of pain – this is
anecdotally described as the single most important
clinical finding for GCA.
Systemic Exam
• Other clinical features include:
– Pulselessness and/or tenderness and
inflammation along the course of the temporal
artery and bruits in the cranial or neck area
– Jaw claudication
– Atrophy of temporal and tongue muscles
– Temporal artery blood flow measurements may
reduced
Systemic Exam
• Cerebrovascular disease occurs in 1-25% of
patients and is believed to be the most
common cause of death in patients with
GCA.
• Neurologic problems associated with GCA
include myopathy, neuro-otologic
syndromes, neuropsychiatric syndromes,
peripheral neuropathies, and seizures.
Systemic Exam
• Cardiovascular, pulmonary, gastrointestinal,
renal, and dermatologic manifestation also
may occur.
Causes
Causes
• Overall, etiology is unknown.
• Genetic – persons of northern European
decent.
• Infectious – Chlamydia and parvovirus
may be impetus for destructive
inflammation.
Causes
• Autoimmune – (cellular and humoral). The
granulomatous histopathology of GCA has
suggested the presence of a cell-mediated
immune reaction directed at antigens in or
near elastic tissue in the arterial walls.
Differentials
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Polymyalgia rheumatica
Transient ischemic attack
Systemic infections
Amyloidosis with prominent vascular involvement
Neoplasms
Arteriosclerotic vascular disease
Arteriovenous fistulas
Other forms of vasculitis
Lab Studies
Lab Studies
• Erythrocyte sedimentation rate (ESR) (moderate
to > 100 mm/h) is common and is rarely (~ 3%)
normal. Highly elevated ESR results are
characteristic of a GCA process.
• C-reactive protein (CRP) – elevated and reflects
the underlying inflammatory process. May assist
in monitoring for treatment dosing and response.
Lab Studies
• Fibrinogen –increased along with other acute
phase reactants.
• Most patients are mildly anemic (normochromic,
normocytic) during the active phases. Platelet
counts often are increased.
• Hepatic enzymes (alkaline phosphatase and serum
aspartate aminotransferase [SGOT]) are elevated
in 20-30% of cases.
• Prolonged prothrombin time also may be found.
Imaging Studies
Imaging Studies
• Aortic arch and cerebral angiography may
show occlusion or alternating stenotic areas.
• Arteriography is sensitive, but nonspecific
and is deemed unreliable diagnostically.
• CT and MRI imaging of the brain are not
first-line diagnostic procedures for GCA.
Other Tests
• Automated visual field typically reveals an
inferior altitudinal defect, inferior nasal
sectorial defect, or central scotoma.
Procedures
• Superficial temporal artery biopsy (TAB)
shows focal granulomatous arteritis, often
with giant cells with skip areas within
normal arterial walls.
Histologic Findings
Histologic Findings
• Early cases:
– Collections of lymphocytes are confined to the
region of the internal or external elastic lamina
or adventitia in early cases or regions show
arteries with minimal involvement.
– Intimal thickening, with prominent cellular
infiltration, is typically present.
Histologic Findings
• Late cases:
– All layers are affected in late cases or regions
of arteries with marked involvement.
– Widespread areas of necrosis of portions of the
arterial wall.
– Elastic laminae usually involved.
– Some plasma cells and fibroblasts are usually
present.
Treatment
Medical Care
• The universally accepted treatment for GCA
is high-dose corticosteroid therapy.
• Goals of treatment are to reverse the disease
and to prevent further progression. This is
of utmost importance especially in the
ophthalmic arteries to prevent blindness.
Medical Care
• Using constitutional symptoms, vascular
symptoms, and the ESR findings as guides,
physicians usually are able to gradually
taper steroids to a maintenance dose for 2
years.
• Initially, high doses of corticosteroids may
be given at 1-2 mg/kg/day until the disease
activity is suppressed adequately.
Medical Care
• Once the signs of clinical inflammation are
suppressed and the ESR is maintained at a low
level, corticosteroids may be tapered slowly.
• No agreement exists as the length of treatment
with corticosteroids for GCA.
• It may be reasonable to maintain the patient on
treatment for 2 years to lessen the chances for
relapses, although relapses have been reported.
Surgical Care
• Aside from the performance of a superficial
TAB, usually no further surgical
intervention is necessary in the management
of patients with GCA.
Consultations
• Rheumatologist
– Indicated when initiating high-dose steroid
therapy for presumed GSA.
– Indicated to consider other forms of
immunosuppressive therapy in those patients
who do not respond adequately to steroid
therapy.
Follow-up
Further Inpatient Care
• Monitor the patient’s symptoms and the
subjective and objective visual acuity on a
daily basis.
Further Outpatient Care
• Since this is a potentially blinding and lethal
disease, regular follow-up care after a
successful initial management of an acute
process is considered a standard of care.
In/Out Patient Medications
• Corticosteroids are the mainstay of therapy.
In some steroid-resistant cases, a recipe of
cyclosporin-azathioprine or cyclosporinmethotrexate may be used.
Complications
• Reported vascular complications include
stroke, aortic artery aneurysms, myocardial
infarction, and visceral organ ischemia.
• The complications associated with late
disease are rare, but attempts to discontinue
therapy often cause relapses.
Prognosis
• GCA is a chronic disease that may last for
years. Although the overall course of the
disease is one of progressive improvement,
and eventual complete resolution, the
clinical course is highly variable, and, in
some patients, it may be protracted for
months to years.
Patient Education
• Instruct patients to immediately consult a
physician when they experience symptoms
of transient blurring of vision because of the
possibility of impending attacks of GCA.
• Discuss the adverse effects of corticosteroid
therapy so that patients will use prednisone
carefully and as instructed.
Competency Exam
Question One
The following arteries are most commonly
involved in GCA, except:
A) Extracranial vertebral arteries
B) Posterior ciliary arteries
C) Ophthalmic arteries
D) Intracranial arteries
E) Superficial temporal arteries
Question One
The following arteries are most commonly
involved in GCA, except:
A) Extracranial vertebral arteries
B) Posterior ciliary arteries
C) Ophthalmic arteries
D) Intracranial arteries
E) Superficial temporal arteries
Question Two
True or False: Giant Cell Arteritis mostly
affects those from the African-American
and Asia descent.
Question Two
True or False: Giant Cell Arteritis mostly
affects those from the African-American
and Asia descent.
Question Three
What is the universally accepted treatment for
Giant Cell Arteritis:
A) Azathioprine
B) Cyclophosphamide
C) High-dose corticosteroids
D) Dapsone
E) Methotrexate
Question Three
What is the universally accepted treatment for
Giant Cell Arteritis:
A) Azathioprine
B) Cyclophosphamide
C) High-dose corticosteroids
D) Dapsone
E) Methotrexate
End of Lecture
Questions?