Rosen & Barkin's 5-Minute Emergency Medicine Consult (298 page)

Genetic predisposition is linked to HLA-DR4—60% prevalence

• Unknown
  
• Genetic, enviromental and autoimmune factors have been identified
  

DIAGNOSIS

• Presence of any 3 or more of the following in patients with vasculitis:
  
  • ESR >50
    
  • Age greater than 50 yr
    
  • New onset of localized headache
    
  • Tenderness or decreased pulsation of temporal artery
    
  • New visual symptoms
    
  • Biopsy revealing necrotizing arteritis
    

• 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 common symptom (50%)
  
• Constitutional symptoms:
  
  • Fatigue
    
  • Malaise
    
  • Anorexia
    
  • Weight loss
    
  • Weakness
    
  • Arthralgias
    
  • Low-grade fever
    
• Visual findings:
  
  • Findings are usually in 1 eye
    
  • May develop weeks to months after the onset of other symptoms
    
  • May fluctuate, but visual impairment does not usually improve over time, even with treatment
    
  • Amaurosis Fugax
    
  • Blindness
    
  • Diplopia
    
  • Ptosis
    
  • Extraocular muscle weakness
    
  • Scotomata
    
  • 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
  
• 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 one-third 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
  

• 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
  

• Elevated ESR, often >100 mm/hr
  
  • ESR <40 is rare
    
• 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
  

• Doppler ultrasound:
  
  • Decreased blood flow in temporal, facial, and ophthalmic arteries
    
  • Presence of a halo is highly suggestive of active TA.
    
• MRI:
  
  • Indicated for exam of large arteries
    
• Angiogram:
  
  • Smooth, tapered occlusions or stenosis
    

• Temporal artery biopsy:
  
  • Multiple sections should be done in as soon as feasible after initiation of steroid therapy
    
  • Gold standard for diagnosis
    
  • Contralateral biopsy is recommended if 1st is negative and index of suspicion high
    

• 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)
  

TREATMENT

• Acute symptoms may be confused with stroke
  
• Initiate appropriate monitoring and oxygen
  
• Patients may be hypotensive from 1 of the rare sequelae (aortic dissection, abdominal aortic aneurysm, or myocardial infarction)
  

Although rare, patients may present with vascular catastrophe such as aortic dissection or myocardial infarction and need appropriate aggressive early management

• Steroids:
  
  • Strong clinical indications should be present if started before temporal artery biopsy
    
  • Scheduling of TA biopsy should not delay steroid therapy when clinical suspicion is high as pathologic vessel changes resolve slowly and will remain for weeks
    
  • 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
  

• 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 1st 1–3 days
  
• Low-dose aspirin therapy to reduce thrombotic risks
  
• Pain management with NSAIDs or narcotics
  

FOLLOW-UP

• Patients with impending vascular complications or acute focal neurologic findings
  
• Patients with associated acute visual loss
  

• Less symptomatic patients without evidence of end-organ involvement
  
• Follow-up arranged within 1--2 days
  

• Rheumatology
  
• Ophthalmology if associated with visual symptoms
  
• Neurology with acute focal neurologic findings
  

• Rheumatology for steroid management and search for associated connective tissue disorders
  
• Ophthalmology and neurology for visual disturbance and/or focal neurologic findings.
  

• Permanent visual loss is the most feared complication of GCA
  
• Do not delay initiation of steroid therapy to await biopsy if strong clinical suspicion for GCA exists or if visual changes are reported
  
• Jaw claudication and amaurosis fugax, while often dramatic, are symptoms patients often neglect to report. Query directly and specifically about them if TA/GCA is being considered
  
• 25–50% of patients who present with acute loss of vision in 1 eye who go untreated will develop bilateral blindness
  

• Firestein SG, Budd RC, Gabriel SE, et al., eds.
  Kelley’s Textbook of Rheumatology.
  9th ed. Philadelphia, PA: Saunders Elsevier; 2013.
  
• Mackie SL, Pease CT. Diagnosis and management of giant cell arteritis and polymyalgia rheumatic: Challenges, controversies, and practical tips.
  Postgrad Med J.
  2013;89:284–292.
  
• Nordborg E, Nordborg C. Giant cell arteritis: Strategies in diagnosis and treatment.
  Curr Opin Rheumatol
  . 2004;16:25–30.
  
• Waldman CW, Waldman SD, Waldman RA. Giant cell arteritis.
  Med Clin North Am
  . 2013;97:329–335.
  

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