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Giant Cell Arteritis (GCA) (Temporal Arteritis), Emergency Medicine


Basics


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
  • 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
  • 2 to 4 times more common in women
  • Rare in African American patients, common in Whites
  • There is a strong association with polymyalgia rheumatica (PMR) ~50%

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

Etiology


  • 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

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 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

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

Diagnosis Tests & Interpretation


Lab
  • 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

Imaging
  • 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

Diagnostic Procedures/Surgery
  • 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

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)

Treatment


Pre-Hospital


  • 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)

Initial Stabilization/Therapy


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

Ed Treatment/Procedures


  • 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

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

Follow-Up


Disposition


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

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

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.

Pearls and Pitfalls


  • 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

Additional Reading


  • Firestein  SG, Budd  RC, Gabriel  SE, et al., eds. Kelleys 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.

See Also (Topic, Algorithm, Electronic Media Element)


  • Aortic Dissection, Thoracic
  • Central Retinal Artery Occlusion
  • Central Retinal Vein Occlusion
  • Glaucoma
  • Systemic Lupus erythematous
  • Vasculiits

Codes


ICD9


446.5 Giant cell arteritis  

ICD10


  • M31.5 Giant cell arteritis with polymyalgia rheumatica
  • M31.6 Other giant cell arteritis

SNOMED


  • 414341000 giant cell arteritis (disorder)
  • 239938009 Giant cell arteritis with polymyalgia rheumatica (disorder)
  • 400130008 Temporal arteritis (disorder)
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