Temporal arteritis

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By James Goodwin MD

Temporal arteritis is also known as or subsumes Bagratuni syndrome, Cranial arteritis, Giant cell arteritis, Polymyalgia arteritica, and Rumbold disease. -ed.

Temporal arteritis is an important cause of ischemic complications in elderly patients and timely diagnosis can prevent important morbidity, most notably blindness from either anterior ischemic optic neuropathy or central retinal artery occlusion. These patients, left untreated, are also at increased risk for ischemic stroke. Treatment with high-dose corticosteroids is effective in preventing these complications, and initial treatment with intravenous pulse corticosteroids leads to more sustained clinical remission and lower subsequent oral corticosteroid dosage requirement. Other adjunctive immunosuppressant drugs are also currently being used in this setting as steroid-sparing agents. In this clinical summary, Dr. James Goodwin of the University of Illinois Eye & Ear Infirmary cites a large prospective multicenter European study showing that the sensitivity and specificity of contrast enhanced magnetic resonance imaging of the superficial cranial arteries compared favorably with the clinical diagnosis and the temporal artery biopsy diagnosis when available, and a similar study emphasized the frequency of intradural arterial enhancement, particularly in the internal carotid system.

Key points

         • Temporal arteritis is a systemic vascular disease in which inflammation, usually with giant cells, affects the internal elastic lamina, adventitia, and adjacent media of medium and large arteries with a predilection for those in the head and neck.    • It is a disease almost exclusively in the elderly (older than 60 years) and usually presents with headache along with manifestations of ischemia in tissues of the head including most commonly pain, ulceration, and necrosis involving the scalp and oral mucosa.

          • Ischemia of the temporalis and masseter muscles produces a distinctive syndrome called jaw claudication with crescendo pain in the mandibular region while chewing solid food and gradual relief after cessation of muscular activity in the involved muscles.
          • The most common serious complication is vision loss from ischemic optic neuropathy or central retinal artery occlusion.
          • Ischemic stroke can occur secondary to emboli from involved extracranial carotid and vertebral arteries—intracranial segments of the arteries are not involved, possibly because they lack an internal elastic lamina.
          • Serious complications can be prevented by treatment with systemic corticosteroids in adequate doses over prolonged periods.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited