Chapter 47 Temporal arteritis (giant cell arteritis, GCA) is a systemic disorder of unknown etiology characterized by an inflammatory obliterative vasculitis of both medium and large arteries. Branches of the external carotid and ophthalmic arteries are particularly (but not exclusively) involved.1 The most commonly affected vessels are the superficial temporal, vertebral, ophthalmic, and posterior ciliary arteries. It primarily affects patients over the age of 60. For further information, see Chapter 19. Temporal arteritis causes permanent visual loss in ~20% of affected patients.2 There is a high (~75%) probability of the second eye being involved following disease in the first eye.3 Understandably, early diagnosis and prompt treatment are essential. • Arteritic anterior ischemic optic neuropathy (arteritic AION) • Caused by presumed occlusion of short posterior ciliary arteries4–6 • Patient usually presents with sudden onset of decreased vision, decreased color vision, a relative afferent pupillary defect, and visual field loss consistent with optic nerve disease. • Optic nerve head shows disc edema, often chalky white in color, with nerve fiber layer hemorrhages, and cotton wool spots. • The other eye is frequently involved, with its most “at risk” period soon after the first eye’s involvement. • Transient monocular vision loss • Usually precedes permanent visual loss from arteritic AION2. • Presumably reversible ischemia to the posterior ciliary circulation, which when prolonged causes arteritic AION • Ophthalmoplegia Diplopia due to presumed ischemia of an extraocular muscle or its nerve3. • Central retinal artery occlusion Occurs in 10% of patients who have visual loss from temporal arteritis • Branch retinal artery occlusion • Rarely iritis, conjunctivitis, scintillating scotoma, anterior segment necrosis, tonic pupil. and ocular hypotony • Headache The most common manifestation of temporal arteritis7. • Usually severe • Scalp tenderness • Patient may be unable to sleep on affected side, or unable to comb hair without significant discomfort. • Etiology presumably due to ischemia • Jaw claudication • Due to ischemia of muscles of mastication • One of the strongest predictors of temporal arteritis and a positive temporal artery biopsy8 • Anorexia, weight loss, fever, malaise, anemia The diagnosis of GCA should be considered in all individuals over 55 years of age with headache, scalp tenderness, jaw claudication, shoulder pain, and transient or permanent visual loss or diplopia. In the study of Vilaseca and coworkers,9 the simultaneous presence of recent onset headache, jaw claudication, and abnormalities of the temporal arteries on physical examination had a specificity of 95% for the diagnosis of GCA. Initial diagnostic tests to be performed for laboratory confirmation include erythrocyte sedimentation rate (ESR) and C-reactive protein. Nonetheless, the temporal artery biopsy is the gold standard for diagnosis. • A temporal artery biopsy is the gold standard for diagnosing GCA. • A positive biopsy should be obtained to justify placing an elderly patient on high-dose, long-term daily steroids. • Biopsy has a 95% sensitivity and 100% specificity for the diagnosis of GCA.10 Although the patient at presumed risk should be started on high-dose daily steroids as soon as the diagnosis of GCA is considered, plans for doing the biopsy must be put in place immediately, and the biopsy done within 7 to 10 days. • Some practitioners routinely perform bilateral temporal artery biopsy. Most, however, initially biopsy one side, generally the side with the visual symptoms. • Biopsy findings can be affected by systemic steroid therapy, but usually remain positive for at least 7 to 14 days after steroid treatment is initiated.11 • The temporal artery is one of the major terminal branches of the external carotid artery, the other terminal branch being the internal maxillary artery. • The superficial temporal artery begins in the parotid gland and generates the transverse facial artery, which supplies the parotid glands.
TEMPORAL ARTERY BIOPSY
CLINICAL FEATURES OF TEMPORAL ARTERITIS
OCULAR MANIFESTATIONS
NONOCULAR MANIFESTATIONS
THE DIAGNOSIS OF TEMPORAL ARTERITIS
TEMPORAL ARTERY BIOPSY
RELEVANT ANATOMY12