Giant Cell Arteritis

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Findings

  • Temporal headaches
  • Tender temporal artery, possibly indurated -> no palpable pulse!
  • Jaw claudication (pain during chewing)
  • Scalp hypersensitivity/pain (when combing)
  • Double vision
  • Shoulder/hip stiffness/pain
    • Associated with polymyalgia rheumatica
  • Amaurosis fugax
  • Sudden loss of vision
  • Possibly nonspecific symptoms like neck pain, weight loss, fever, night sweats, discomfort, depression

Examination

  • Complete ophthalmological assessment including:
    • Visual acuity
    • Pupil examination: Relative afferent pupillary defect (RAPD)?
    • Motility testing
    • Confrontational visual field (finger counting)
    • Palpation of temporal artery (tenderness, hardening, palpable pulse?)
    • Fundus examination: Optic disc assessment

Manifestations

  • Arteritic anterior ischemic optic neuropathy (A-AION): optic disc swelling, pale (chalky white)
  • Central retinal artery occlusion: Cherry-red spot (often combined with posterior ciliary artery occlusion -> consider fluorescein angiography)
  • Amaurosis fugax -> Transient ischemic attack (TIA)
  • Ocular ischaemic syndrome: Rare, due to involvement of ophthalmic artery
  • Diplopia: Caused by ischemia of ocular motor nerves or extraocular muscles; transient or constant

Work-up

  • Laboratory tests: ESR (upper normal limit: age/2 for men, (age + 10)/2 for women), CRP, blood count (anemia, thrombocytosis)
  • Duplex sonography of brain-supplying vessels (angiology) promptly: Halo sign (edema in vessel wall)? Compression signs? Also inquire about flow reversal (retrograde flow) in ophthalmic artery (for later biopsy)?
  • Plan a temporal artery biopsy
    • Primarily ipsilateral to the affected eye; extract at least 2 cm of the artery
    • Ideally, perform rapidly, recommended within 7-10 days after initiating steroid therapy
  • Possibly in the course: MRI, angiography, FDG-PET

Treatment

  • Goal: Prevent blindness in the other eye!
  • Hospitalization
  • High-dose steroid therapy:
    • Start immediately if highly suspected (before completing diagnostics!)
    • Initial Solumedrol 4x250mg i.v./d for 3 days
      • Blood pressure and blood sugar monitoring
    • From day 4: Oral prednisone 1mg/kg for 1 month, then taper slowly
      • Duration of therapy 1-2 years (at least 6 months), possibly requiring long-term therapy
    • Additionally: Pantoprazole 40mg 1x/d + Calcimagon D3 2x/d
  • Aspirin Cardio 100mg 1x/d
  • Actemra therapy (Tocilizumab) in consultation with a rheumatologist
    • in case of signs of activity/recurrence despite steroid therapy or instead of long-term steroid therapy
    • CAUTION: CRP may no longer be reliable under Actemra!
  • If no initial eye involvement, consider refraining from high-dose intravenous steroid therapy
    • Start with oral prednisone 1 -1.5 mg/kg body weight daily

Ophthalmologic Follow-up

  • After discharge:
    • Outpatient follow-up after approximately 1-2 weeks, then individually (e.g., after 2, 4, 12 weeks; then every 3 months)
  • After steroid tapering, close monitoring for at least 1 year is advisable

Sources

  • EyeWiki Giant Cell Arteritis
  • EyeWiki Temporal Artery Biopsy
  • C Turesson, O Börjesson, K Larsson, AJ Mohammad & A Knight (2019) Swedish Society of Rheumatology 2018 guidelines for investigation, treatment, and follow-up of giant cell arteritis, Scandinavian Journal of Rheumatology, 48:4, 259-265, DOI: 10.1080/03009742.2019.1571223
  • Hellmich B, Agueda A, Monti S, et al2018 Update of the EULAR recommendations for the management of large vessel vasculitis Annals of the Rheumatic Diseases 2020;79:19-30.
  • UpToDate – Treatment of giant cell arteritis
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition(2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)