Transcript Giant Cell Arteritis

Giant Cell Arteritis
Julie Story
July 27, 2006
Overview
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Typical case presentation
Differential diagnosis
Confirming the diagnosis
Associated symptoms & conditions
Treatment
Monitoring
The frontier
Classic Case Presentation
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55 yo female (70%)
Headache (50-75%)
Fever (50%)
Jaw pain with chewing (50%)
Fatigue (40%)
Monocular vision loss (15%)
Non-productive cough (10%)
Bilateral vision loss (5%)
Arm claudication (3-15%)
Arthritis Foundation 2001, Givre
2006, Hunder 2006.
Differential Diagnosis
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Migraine
Infection
Tempo-Mandibular Joint disorder
Ischemia
– Carotid stenosis or dissection
– Atherothrombosis or embolus
• Vasculitis
– Giant cell arteritis
– Takayasu’s arteritis
– Wegener’s granulomatosis
• Orbital mass
• Papilledema
• Demyelination of optic nerve
American College of Rheumatology
GCA Classification Criteria
• 3 of the following:
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≥ 50 yo at disease onset
Localized new headache
Tenderness or decreased temporal artery pulse
ESR > 50 mm/hr
Arterial biopsy with necrotizing arteritis with
mononuclear cell predominance or granulomatous
process with multinucleated giant cells
• 94% sensitive, 91% specific
Confirming GCA Diagnosis
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History
Physical exam
Labs
Temporal artery biopsy
Ultrasound not consistently helpful
Good response to prednisone
Physical Exam
• Tender, thickened temporal arteries
• Carotid bruits
• Limited ROM of neck, shoulders, and hips
Labs
• ESR > 100 mm/hr
• Acute phase reactants
– Increased CRP
– Increased fibrinogen
– Decreased albumin
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Anemia
Microscopic hematuria
Increased AST and alk phos
Elevated factor VIII and Von Willebrand factor
Elevated IL-6
Arthritis Foundation Primer on the
Rheumatic Diseases. Edition 12.
Temporal Artery Biopsy
• Best yield w/ 3-5cm samples bilaterally
– 94% Sensitive
– Positive biopsy indicates poorer prognosis
– Bilateral biopsy only increases sensitivity by
2-3%
Arthritis Foundation Primer on the
Rheumatic Diseases. Edition 12.
Temporal Artery Biopsy
• Before or after treatment?
– Treatment with steroids does not significantly alter biopsy results
when samples are taken within 6 weeks.
• Small sample size (11 pts in 3 groups), but
• Risk of vision loss while waiting for biopsy is high
• Is a biopsy really necessary?
– 5%-9% false negative due to skip lesions
– Risk of scalp necrosis, stroke, facial nerve injury, infection,
bleeding, pain
– Results rarely change treatment
– If ≥ 3 ACR criteria are present without biopsy, biopsy is
unnecessary
– Biopsy is more helpful in atypical cases or when only 2 criteria
are otherwise met
Davies C et al. Temporal artery
biopsy...Who needs one?
Associated Conditons
• Polymyalgia rheumatica
– 40-50% of patients w/ GCA also have PMR
– 15% of patients w/ PMR have GCA
– Stiff, sore muscles in shoulders and hip girdle
– Worse in the morning
– ESR > 40mm/hr
– Fever (10-15%)
• Thoracic aortic dissection
• Increased risk of cancer?
Hunder G. Clinical Manifestations
and diagnosis of giant cell
Treatment of GCA
• Start glucocorticoids immediately
– 40-60 mg prednisone daily; Increase if suboptimal response
– If visual loss, consider IV steroids
– Maintain dose until sx remit and ESR and other labs return to normal,
then taper 10% every 2 weeks as tolerated
– If no response, perhaps it’s not GCA
• Methotrexate + glucocorticoids may prevent relapses and
spare some steroid side effects, but results are not consistent
• Low-dose aspirin is recommended to reduce the risk of visual
loss and CNS effects. Consider PPI to protect GI tract
• DEXA, Calcium and Vitamin D to protect against steroidinduced osteoporosis
Arthritis Foundation 2001,
Hoffman 2002, Jover 2001,
Monitoring
• Mostly symptomatic
• Vision rarely decreases after treatment
• Increased risk of thoracic aortic aneurysm
– 17-fold increase, but still rare
– Chest X-ray annually for 10 years
New Frontiers
• Diagnosis w/ MRI of temporal artery
– Abnormal contrast enhancement
– $$$, limited availability
• Further study of methotrexate use in GCA
treatment needed
• TNF-α Inhibitors
– Study underway
Lamprecht P. TNF-alpha inhibitors
in systemic vasculitides and
References
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Arthritis Foundation Primer on the Rheumatic Diseases. Edition 12. 2001.
Davies C et al. Temporal artery biopsy... Who needs one? Postgrad Med J. 2006; 82:
476-478.
Givre S, Van Stavern G. Amaurosis fugax: transient monocular and binocular vision
loss. UpToDate. 2006.
Hoffman G et al. A multicenter, randomized, double-blind, placebo-controlled trial of
adjuvant methotrexate treatment for giant cell arteritis. Arthritis & Rheumatism. 2002;
46: 1309-1318.
Hunder G. Clinical Manifestations and diagnosis of giant cell (temporal) arteritis.
UpToDate. 2006.
Hunder G. Treatment of giant cell (temporal) arteritis. UpToDate. 2006.
Image: Man with headache. www.arc.org.uk/about_arth/booklets/6047/6047.htm.
2006
Image: PMR pain. www.allaboutarthritis.com/.../PMR300X300.jpg. 2006.
Image: Temporal artery. www.uveitis.org/medical/articles/case/gca.html. 2006
Image: Temporal artery biopsy. www.hopkinsmedicine.org/gec/studies/gca.html .
2006.
Jover J et al. Combined Treatment of giant-cell arteritis with methotrexate and
prednisone. Ann Intern Med. 2001; 134: 106-114.
Kupersmith M et al. Visual performance in giant cell arteritis (temporal arteritis) after 1
year of therapy. Br J Ophthalmol. 1999; 83: 796-801
Lamprecht P. TNF-alpha inhibitors in systemic vasculitides and connective tissue
diseases. Autoimmunity Reviews. 2005; 4: 28-34.
Ray-Chaudhuri N et al. Effect of prior steroid treatment on temporal artery biopsy
findings in giant cell arteritis. Br J Ophthalmol. 2002; 86: 530-532.