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temporal arteritis; cranial arteritis; giant cell arteritis; Horton's disease

More appropriately giant cell arteritis as some patients may have arteritis confined to the great arteries. Etiology: 1) unknown 2) often associated with polymyalgia rheumatica (PMR) - may occur despite low-dose glucocorticoids used for treatment of PMR 3) evidence of Herpes zoster found in temporal arteries of 74% of cases [17] Epidemiology: 1) occurs in elderly patients a) average age at diagnosis is 72 years of age b) rare in individuals < 50 years of age 2) male:female ratio is 2:1 3) rare in blacks Pathology: 1) inflammation of medium & large sized arteries 2) generally involves one or more branches of the carotid artery, especially superficial temporal artery 3) less commonly, branches of the thoracic aorta & abdominal aorta are affected 4) infiltrative granuloma with giant cells 5) disruption of internal elastic membrane 6) intimal proliferation 7) thrombosis 8) disease may be systemic 9) Herpes zoster antigen most frequently located within "skip areas" with giant cell vasculitis adjacent to these areas [17] Clinical manifestations: 1) new onset headache, may be localized [7] - temporal or atypical headache [5] 2) jaw claudication or tongue claudication - sensitivity 50%, highest diagnostic specificity [41] - also see diagnostic criteria (below) 3) visual changes (involvement of ophthalmic artery) a) acute monocular vision loss b) diplopia (may be binocular) [7] c) amaurosis fugax (most predictive of blindness) [41] d) visual field defects e) unilateral scotoma f) may be transient (5 minutes) 4) ophthalmoplegia a) non-specific motility deficits b) pupils normal 5) ptosis 6) retinal or optic disk ischemia (swelling) 7) neurologic deficits a) mononeuritis multiplex b) delirium c) dementia d) transient ischemic attack e) stroke 8) polymyalgia rheumatica 9) weight loss 10) malaise, fatigue 11) fever (may present as fever of unknown origin) 12) temporal artery tenderness & erythema (2/3)* 13) decreased temporal artery pulsation 14) nodules over the temporal artery [9] 15) bruits in head & neck 16) diminished pulses in upper extremities - new onset arm claudication - differential blood pressure in arms [41] 17) systemic inflammatory signs a) synovitis b) pericarditis c) aortitis, heart failure [5] 18) scalp tenderness & rarely necrosis 19) facial pain 20) respiratory symptoms (10%) a) cough (dry) [11] b) sore throat c) hoarseness 21) Raynaud's disease 22) onset may be sudden or gradual 23) initial presentation as ischemic stroke [29] (case report) * do not be dissuaded by negative physical examination findings alone [42] Diagnostic criteria: likelihood ratios [35] - limb claudication (6.0) - jaw claudication (4.9) - temporal artery thickening on biopsy (4.7) or loss of pulse (3.3) - platelet counts > 400,000/uL (3.8) - temporal tenderness (3.1) - erythrocyte sedimentation rate > 100 mm/h (3.1) findings suggesting another diagnosis [35] - erythrocyte sedimentation rate < 40 mm/h - C-reactive protein in serum < 2.5 mg/dL - age 49-70 years [39] Laboratory: 1) increased erythrocyte sedimentation rate [ESR] (85%) a) 100 mm/hr is average value b) ESR frequently > age c) a normal value does not rule out temporal arteritis [30] d) glucocorticoid therapy may normalize ESR & serum CRP [30] 2) increased serum C-reactive protein [serum CRP] (85%) 3) complete blood count (CBC) a) anemia of chronic inflammation may be present b) leukocyte count is generally normal c) platelet count is generally high [9] 4) serum alkaline phosphatase may be mildly elevated 5) serum AST & serum ALT may be mildly elevated [7] 5) testing for Herpes in temporal artery biopsy tissue - Herpes zoster antigen (detected in 74%) [17] - Herpes zoster DNA (detected in 40%) [17] 5) see ARUP consult [10] Special laboratory: - temporal artery biopsy a) biopsy should not delay glucocorticoid therapy [5] - results of biopsy not affected by 2-5 days of prednisone therapy [3] - biopsy specimens remain interpretable for at least 2 weeks after initiation of prednisone [5] - 6 weeks of prednisone therapy may not affect results of biopsy [43] b) biopsy segment should be > 5 cm c) ultrasound-guided biopsy of contralateral temporal artery 2-5 days after initiation of steroid therapy if initial biopsy is negative [5,12] d) prior to biopsy, check colateral circulation with 1-2 min occlusion e) frozen section examination of an initial biopsy determines need for bilateral biopsies [36] f) bilateral biopsy would net an estimated additional 5% of diagnoses [40] Radiology: - imaging may have a role in diagnosis of temporal arteritis, but are not yet gold standard. - superficial temporal artery ultrasound - 77% sensitivity & 96% specificity for diagnosis of giant cell arteritis (halo sign) [39] - bilateral halo sign may confirm diagnosis making biopsy unnecessary [48] - temporal artery ultrasound biopsy alternative in centers with expertise [38] - CT angiography of neck & thorax if giant cell arteritis suspected, but bilateral temporal artery biopsy negative - magnetic resonance angiography (MRA) may be used rather than CT angiography [5] - magnetic resonance imaging (MRI) of scalp arteries [27] - may reveal isolated occipital artery involvement [27] - MRI has 73% sensitivity & 88% specificity for diagnosis of temporal arteritis - 18F-FDG PET/CT sensitivity of 82% & specificity of 100% [34] - annual or regular screening with chest C-ray, CT angiography, of MR angiography may be indicated (GRS9) [7] Differential diagnosis: 1) Takayasu's arteritis 2) Wegener's granulomatosis [44] cANCA in serum (90%) & pANCA in serum 3) polyarteritis nodosa 4) hypersensitivity 5) vasculitis 6) atherosclerosis 7) amyloidosis 8) malignancy 9) other causes of: fever, headache, blindness, myalgias, arthralgias Complications: 1) ischemic optic neuritis 2) visual loss (irreversible) resulting from from necrosis of the posterior ciliary branch of the ophthalmic artery 3) compression fractures (30%) 4) thoracic aortic aneurysm & aortic dissection (11%) [7] - median time to diagnosis is 5.8 years 5) aortic regurgitation & congestive heart failure 6) ischemic stroke [8], especially vertebrobasilar stroke [24,41] 7) vascular dementia [8] 8) peripheral artery disease 9) myocardial infarction [5] 10) tongue necrosis due to thrombosis of lingual artery [31]* * image (tongue necrosis) Management: 1) refer to ophthalmologist [7] 2) glucocorticoids (prednisone) a) 40-60 mg QD divided BID-QID (oral noninferior to IV) [32,37] b) change to QD schedule after clinical response c) taper when symptoms have resolved & ESR has normalized 1] reduce dose by 10% q2-4 weeks to 15-30 mg QD 2] maintain 15-30 mg QD for several months 3] reduce dose by 2.5 mg q2-4 weeks to 5-7.5 mg QD 4] maintain 5-7.5 mg QD for several months 5] reduce dose by 1 mg q 1-3 months d) alternate day therapy not effective in initial treatment e) response to therapy with 3-7 days f) ESR normalizes in 2-4 weeks g) duration of treatment is generally 1-2 years; however, some patients may need up to 5 years of therapy h) 26 week glucocorticoid taper when used with tocilizumab [37] - tocilizumab [21,22,28] 162 mg SQ weekly or every other week combined with 26 week prednisone taper [37] preferred initial treatment [5,38] i) relapse in 34% of patients [19] 1] 73% of relapses occur during glucocorticoid taper 2] most common relapse symptoms a] headache (42%) b] polymyalgia rheumatica (51%); 3] ischemic symptoms (29%) a] limb claudication b] jaw or tongue claudication c] visual impairment [19] 4] ESR & serum CRP normal in 18% of relapses [19] 5] concurrent immunosuppressive agents (tocilizumab) in addition to glucocorticoids might increase risk of relapse [5,19] j) treatment decisions should be based on symptoms, hemoglobin & ESR 3) visual loss is a medical emergency a) IV methylprednisolone for recent visual loss (< 36 hours) b) high dose glucocorticoids will prevent blindness c) reversal of blindness is rare 4) alternative agents a) methotrexate is not steroid-sparing b) infliximab is not steroid sparing c) dapsone, azathioprine, cyclophosphamide suggested in some sources, but not mentioned in ref [5] (2009) 5) temporal artery biopsy a) within first 1-2 weeks of glucocorticoid therapy b) glucocorticoid therapy should NOT be held pending biopsy c) unilateral biopsy is positive in 85% of patients, bilateral biopsy is positive in 95% 6) aspirin may lower risk for cerebral ischemia [5] 7) treatment of osteoporosis 8) patient education a) prognosis with treatment is good b) no difference in survival from general population c) blindness rarely occurs during treatment d) relapse occurs in > 50% of patients in the 1st year 5e) relapse after completion of therapy is uncommon

Interactions

disease interactions

Related

anemia of chronic inflammation C-reactive protein (CRP) in serum/plasma erythrocyte sedimentation rate (ESR) polymyalgia rheumatica (PMR) superficial temporal artery

General

arteritis autoimmune disease

Properties

THERAPY: prednisone

Database Correlations

OMIM 187360

References

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