Author:
Donald J.Lefkowits
Description
- Chronic vasculitis of large- and medium-sized vessels that occurs among individuals over 50 yr of age
- Often referred to as temporal arteritis (TA)
- Median age of onset is 72 yr
- Most commonly causes inflammation of arteries originating from the arch of the aorta
- Although usually clinically silent, involvement of the thoracic aorta occurs in a significant minority of patients, and aortic aneurysm or dissection may result
- Thoracic aortic aneurysm is a late manifestation with an incidence 17 times those without TA
- Abdominal aortic aneurysm is about twice as common in those with giant cell arteritis (GCA)
- Pathologic specimens feature patchy mononuclear granulomatous inflammation resulting in a markedly thickened intima and occlusion of the vessel lumen
- Occlusive arteritis may involve thrombosis of the ophthalmic artery resulting in anterior ischemic optic neuropathy (AION) and acute visual loss:
- Visual symptoms are an ophthalmic emergency
- Inflammation of arteries supplying the muscles of mastication results in jaw claudication and tongue discomfort
- Age is the greatest risk factor:
- Rare in patients <50 yr old
- >90% are >60 yr old
- Prevalence in individuals >50 yr is estimated at 1:500
- Increased prevalence in Northern latitude with highest incidence in those of Scand inavian descent
- 2-4 times more common in women
- Rare in African American, Latino, and Asian patients
- There is a strong association with polymyalgia rheumatica (PMR) ∼50%
Genetics
Genetic predisposition is linked to HLA-DR4 - 60% prevalence
Etiology
- Unknown
- Genetic, environmental, and autoimmune factors have been identified
- Presence of any 3 or more of the following in patients with vasculitis:
- ESR >50
- Age >50 yr
- New onset of localized headache
- Tenderness or decreased pulsation of temporal artery
- New visual symptoms, especially transient monocular visual loss
- Temporal artery biopsy revealing necrotizing arteritis
Signs and Symptoms
- May present with acute, subacute, or chronic symptoms:
- Headache is the single most frequent symptom (70%)
- Often localized, boring, or lancinating in quality
- Often described as unilateral over a temple
- Tongue or jaw claudication upon mastication is the next most common symptom (50%)
- Constitutional symptoms:
- Fatigue
- Malaise
- Anorexia
- Weight loss
- Weakness
- Arthralgias
- Low-grade fever
- Visual findings:
- Monocular
- May develop weeks to months after the onset of other symptoms
- May fluctuate, but visual impairment often does not usually improve over time, even with treatment
- Amaurosis fugax
- Blindness
- Diplopia
- Ptosis
- Extraocular muscle weakness
- Scotoma
- Blurred vision
- Scalp tenderness, especially over the temporal artery
- Pulsations over temporal artery:
- Increased pulsations early in disease
- Decreased pulsations late in the disease
- Erythema, warmth, swelling, or nodules over scalp arteries
- Limb claudication, bruits, or decreased pulses over large arteries
- Sore throat, cough, dysphagia
- Rare findings:
- Respiratory symptoms
- Ischemic chest pain
- Congestive heart failure
- Neurologic problems:
- Occur in up to 1/3 of patients:
- Neuropathies
- Transient ischemic attacks
- Cerebral vascular accidents
- Occult manifestations include:
- Glossitis
- Lingual infarction
- Tongue infarction
- Raynaud phenomenon
- Up to 30% may not present with the classic features of headache, scalp tenderness, visual changes, or jaw claudication
- Frequently associated with PMR (up to 50%):
- Stiffness
- Aching pain in the proximal muscles
- Worse in the morning and decreasing with exercise
- Often associated with synovitis, especially in the knees
Essential Workup
- Focused physical exam with emphasis on:
- Temporal artery and scalp abnormalities
- Complete neurologic exam
- Ophthalmic exam including visual acuity and visual field testing
- Fundoscopy:
- Often normal initially
- Iritis and fine vitreous opacities may be early findings
- Optic nerve edema
- Swollen, pale disc with blurred margins
- Pallor
- Hemorrhage
- Scattered cotton-wool spots
- Vessel engorgement and exudates are seen later
- Any pulse differences in the extremities or bruits over large arteries should be noted
Diagnostic Tests & Interpretation
Lab
- Elevated ESR, often >100 mm/hr
- C-reactive protein above 2.45 mg/dL
- Complete blood count (CBC):
- A mild normochromic anemia is typical
- Thrombocytosis (often mild)
- White cell count can be normal or slightly elevated and differential is usually normal
- Liver function tests and prothrombin time may be elevated; creatine phosphokinase, tests of renal function, and urinalysis are generally normal
- Elevation in interleukin-6 (IL-6) is seen during flares
Imaging
- Color Doppler US:
- Decreased blood flow in temporal, facial, and ophthalmic arteries
- Presence of the “halo sign” is highly suggestive
- MRI:
- Indicated for exam of large arteries
- Angiogram:
- Smooth, tapered occlusions or stenosis
Diagnostic Procedures/Surgery
- Temporal artery biopsy:
- Multiple sections should be done as soon as feasible after initiation of steroid therapy
- Gold stand ard for diagnosis
- Contralateral biopsy is recommended if first is negative and index of suspicion high
Differential Diagnosis
- Vasculitides:
- Polyarteritis nodosa
- Hypersensitivity vasculitis
- Systemic lupus erythematosus
- Takayasu arteritis
- Wegener granulomatosis
- Thrombosis of retinal, ophthalmic, or temporal arteries
- Lyme disease
- Nonarteritic anterior ischemia optic neuropathy (NAAION)
Prehospital
Initial Stabilization/Therapy
- Although rare, patients may present with vascular catastrophe such as aortic dissection or myocardial infarction and need appropriate aggressive early management
- Limb claudication, asymmetric blood pressures, and vascular bruits can point to large vessel involvement
ED Treatment/Procedures
- Steroids:
- Scheduling of TA biopsy should not delay steroid therapy when clinical suspicion is high
- Histopathologic evidence is present for 4-6 wk after initiation of steroid therapy
- Strong clinical indications should be present if treatment started before temporal artery biopsy
- Early, aggressive treatment can significantly reduce the incidence of blindness
- Steroids effectively control systemic and local symptoms within days to weeks
- Treatment with prednisone may continue for years - usual disease length is 3-4 yr
- Symptomatic pain management with NSAIDs, salicylates, and /or narcotics as indicated
Medication
- Prednisone: 60-100 mg PO per day for at least 2 wk before considering tapering
- For acute onset visual symptoms, consider 1,000 mg methylprednisolone IV pulse therapy for the first 3 d
- Low-dose aspirin therapy to reduce thrombotic risks
- Pain management with NSAIDs or narcotics
Disposition
Admission Criteria
- Patients with impending vascular complications or acute focal neurologic findings
- Patients with associated acute visual loss or decrease in visual acuity
Discharge Criteria
- Less symptomatic patients without evidence of end-organ involvement
- Follow-up arranged within 1-2 d
Issues for Referral
- Rheumatology
- Ophthalmology if associated with visual symptoms
- Neurology with acute focal neurologic findings
Follow-up Recommendations
- Rheumatology for steroid management and search for associated connective tissue disorders
- Ophthalmology and neurology for visual disturbance and /or focal neurologic findings
- FiresteinSG, BuddRC, GabrielSE, et al., eds. Kelley's Textbook of Rheumatology. 9th ed.Philadelphia, PA: Saunders Elsevier; 2013.
- HoffmanGS. Giant cell arteritis . Ann Intern Med. 2016;165:ITC65-ITC80.
- JakobssonK, JacobssonL, MohammadAJ, et al. The effect of clinical features and glucocorticoids on biopsy findings in GCA . BMC Musculoskelet Disord. 2016;17:363.
- MackieSL, PeaseCT. Diagnosis and management of giant cell arteritis and polymyalgia rheumatic: Challenges, controversies, and practical tips . Postgrad Med J. 2013;89:284-292.
- WaldmanCW, WaldmanSD, WaldmanRA. Giant cell arteritis . Med Clin North Am. 2013;97:329-335.
- Weyand CM, GoronzyJJ. Clinical Practice. Giant-cell arteritis and polymyalgia rheumatica . N Engl J Med. 2014;371:50-57.
See Also (Topic, Algorithm, Electronic Media Element)
ICD9
446.5 Giant cell arteritis
ICD10
SNOMED
414341000 giant cell arteritis (disorder)
239938009 Giant cell arteritis with polymyalgia rheumatica (disorder)
400130008 Temporal arteritis (disorder)