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large vessel vasculitis which overlaps with polymyalgia rheumatica (PMR).
Histology shows changes which characteristically 'skips' certain sections of affected artery whilst damaging others.
''Features''
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
visual disturbances secondary to anterior ischemic optic neuropathy
tender, palpable temporal artery
features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats
''Investigations''
raised inflammatory markers: ESR > 50 mm/hr (note ESR < 30 in 10% of patients). CRP may also be elevated
temporal artery biopsy: skip lesions may be present
note creatine kinase and EMG normal
''Treatment''
high-dose prednisolone - there should be a dramatic response, if not the diagnosis should be reconsidered
urgent ophthalmology review. Patients with visual symptoms should be seen the same-day by an ophthalmologist. Visual damage is often irreversible