Large Vessel Vasculitides

Snapshot
A 62-year-old woman presents to her primary care physician due to an unremitting headache. She says she has been having this headache for the last few days and is nonresponsive to ibuprofen. Her headache is left-sided and affects the temporal region of her head. She notes to having jaw pain when chewing food or brushing her teeth. On physical exam, there is tenderness to palpation of the left temporal artery. (Giant cell temporal arteritis)

Large Vessel Vasculitides

TypePathologyPresentationStudiesTreatment
Giant cell temporal arteritisIntimal thickeningMedial granulomatous inflammation leading to elastic lamina fragmentation may be focally found, requiring at least a 1 cm segment of the temporal arteryElderly (> 50 years of age) patient with a headache in the temporal region, often worse with chewingPain upon palpation of the temporal artery~50% will present with jaw claudicationAbnormalities in vision may occur (e.g., diplopia or blindness) when the ophthalmic artery is involved May be associated with polymyalgia rheumatica↑ erythrocyte sedimentation rate (ESR)Temporal artery biopsyHigh-dose corticosteroids must be administered immediately in order to prevent blindness
Takayasu arteritisTransmural fibrous thickening of the aortic arch and major vessels Classic in Japanese women who are < 40 years of ageWeak pulse and decreased blood pressure in the upper extremitiesFatigue, malaise, weight loss, and arthralgias↑ erythrocyte sedimentation rate (ESR)Angiography or MRAHigh-dose corticoster