ascending aortic aneurysm with secondary dilatation of the aortic valve. Ocular findings Fundoscopy is indicated in patients with subjective change in visual acuity. In a patient with transient monocular visual loss (TMVL), the ophthalmologic examination can be entirely normal. Some patients have cotton wool spots in the retina, indicative of local, retinal ischemia, depending upon the site of critical vascular lesions. In patients with acute visual loss from arteritic anterior ischemic optic neuropathy (AION), ophthalmoscopic examination shows changes of ischemic optic neuropathy with a swollen pale disc and blurred margins ( picture 2). In patients with permanent visual loss, later findings include optic disc pallor. Fundoscopy can be valuable in differentiating nonarteritic AION from arteritic AION, as occurs in GCA. The affected swollen optic nerve is often pale immediately in GCA, whereas pallor is delayed in nonarteritic AION. The finding of associated retinal or choroidal ischemia in addition to AION is highly suggestive of GCA. Finally, the absence of a crowded optic disc in the unaffected eye of a patient with AION should make the diagnosis of nonarteritic AION unlikely and should increase the probability of arteritic AION [40]. https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 12/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate In posterior ischemic optic neuropathy (PION), acuity is reduced but the appearance of the optic nerve on ophthalmoscopic examination is normal in the acute phase because the ischemic insult has occurred well behind the optic disc. A relative afferent pupillary defect (APD), an objective means of identifying ipsilateral optic nerve dysfunction, will be present unless both eyes have symmetrical optic nerve dysfunction. In PION, the finding of an APD can be a useful sign to implicate optic neuropathy as a probable cause of visual loss even when the optic nerve appears normal. Diplopia is typically transient but if present, can be associated with reduced extraocular movements; diplopia due to a brainstem stroke results in skew deviation. Musculoskeletal findings In patients who also have polymyalgia rheumatica (PMR), active range of motion of the shoulders, neck, and hips can be limited, which in some patients can also be accompanied by distal synovitis, especially affecting the wrists and metacarpophalangeal joints. (See "Clinical manifestations and diagnosis of polymyalgia rheumatica".) LABORATORY FINDINGS Laboratory findings useful in the assessment of giant cell arteritis (GCA) include routine hematologic testing, selected serum chemistry tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). Hematologic abnormalities A normochromic anemia is often present prior to therapy and improves promptly after the institution of glucocorticoids. The anemia is occasionally profound [94]. Many patients also have a reactive thrombocytosis [95,96]. The leukocyte count is usually normal or minimally elevated, even in the setting of widespread systemic inflammation. Erythrocyte sedimentation rate and C-reactive protein A characteristic laboratory abnormality in many patients with GCA is a high ESR, which can reach 100 mm/hour [43,96]. The CRP is nearly always commensurately elevated, though prospective head-to-head studies on the use of the ESR and CRP for the diagnosis and management of GCA are lacking. Among patients with a paraproteinemia or some other cause of a spuriously elevated or depressed ESR, the CRP level is more reliable. (See "Acute phase reactants", section on 'Clinical use'.) Less striking elevations of the ESR [43], however, may also occur. In a population-based study of 167 patients from Olmsted County, 18 (11 percent) had an ESR less than 50 mm/hour and 9 (5 percent) less than 40 mm/hour before treatment was initiated. The patients with an ESR less than 40 mm/hour were less likely to experience systemic symptoms such as malaise, fever, or weight loss; nevertheless, their clinical manifestations, including risk of visual loss, were https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 13/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate indistinguishable from those in patients with a higher ESR [97]. In another series of 173 biopsy- proven cases, 12 patients (5.8 percent) had ESR values less than 46 mm/hour [98]. An additional retrospective study from Olmsted County found normal values for both the ESR and CRP in 4 percent of 177 patients with biopsy-proven GCA at the time of diagnosis [99]. Neither the ESR nor the CRP is a specific biomarker for GCA. Abnormalities in the ESR and CRP can help calibrate the diagnostic probability of GCA, but normal values do not absolve the clinician of the responsibility to pursue the diagnosis in an appropriate clinical setting, nor do marked elevations certify that a diagnosis of GCA is correct. (See "Diagnosis of giant cell arteritis".) Other laboratory abnormalities Elevated serum concentrations of hepatic enzymes, especially the alkaline phosphatase, occur in 25 to 35 percent of patients. The elevations are typically modest and revert to normal with glucocorticoid therapy. The serum albumin level is often moderately decreased at diagnosis but responds quickly to the institution of glucocorticoids. Elevated serum interleukin (IL) 6 concentrations appear to be related closely to clinical disease activity in GCA [100] and may better correlate with clinical relapse than the ESR [101]. However, IL-6 assays are not routinely available, and their clinical utility remains unproven. IMAGING FINDINGS The posterior-anterior (PA) and lateral chest radiograph can show aneurysmal dilation of the ascending aorta, but has limited sensitivity. A detailed discussion of the other imaging modalities used to evaluate the extent of involvement of the aorta and large arteries is presented separately. (See "Clinical manifestations and diagnosis of thoracic aortic aneurysm", section on 'Imaging diagnosis'.) SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Giant cell arteritis and polymyalgia rheumatica".) INFORMATION FOR PATIENTS https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 14/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." th th The Basics patient education pieces are written in plain language, at the 5 to 6 grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more th th sophisticated, and more detailed. These articles are written at the 10 to 12 grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.) Basics topics (see "Patient education: Polymyalgia rheumatica and giant cell arteritis (The Basics)") Beyond the Basics topics (see "Patient education: Vasculitis (Beyond the Basics)" and "Patient education: Polymyalgia rheumatica and giant cell arteritis (Beyond the Basics)") SUMMARY Epidemiology The major risk factor for developing giant cell arteritis (GCA, also known as Horton disease, cranial arteritis, and temporal arteritis) is aging. The disease almost never occurs before age 50 years, and its incidence rises steadily thereafter. Although systemic manifestations are characteristic of GCA and although vascular involvement can be widespread, clinical manifestations of the disease most frequently result from involvement of the cranial branches of arteries originating from the aortic arch. The most sinister complication of GCA, visual loss, is one potential result of the major phenotype of the disease, that of cranial arteritis. (See 'Introduction' above and 'Epidemiology' above.) Clinical features and association with polymyalgia rheumatica The onset of symptoms in GCA tends to be subacute, but abrupt presentations occur in some patients. When taking the patient's history, the clinician must ask about the following types of symptoms: systemic symptoms, such as fever, fatigue, and weight loss; headache; jaw claudication, which is the symptom most highly predictive of a positive temporal artery for the diagnosis of GCA; visual symptoms, particularly transient monocular visual loss (TMVL or amaurosis fugax) and diplopia; and symptoms of polymyalgia rheumatica (PMR). Other symptoms can also be present. (See 'Clinical features' above and 'Association with polymyalgia rheumatica' above.) https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 15/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Ocular involvement A variety of ocular syndromes are associated with GCA. The most common are TMVL and anterior ischemic optic neuropathy (AION). It is estimated that bilateral blindness will develop in 25 to 50 percent of untreated patients who present with loss of vision in one eye. (See 'Permanent vision loss' above.) Large vessel involvement Subclinical involvement of the aorta and large arteries is frequent, a delayed clinical consequence of which can be aortic aneurysm. (See 'Large vessel involvement' above.) Physical examination findings Careful temporal arteries palpation, measurement of the blood pressures in both arms, and assessment of the arterial tree by palpation and auscultation should be performed in all patients with suspected GCA. (See 'Physical examination findings' above.) Laboratory findings Laboratory findings useful in the assessment of GCA include routine hematologic testing, selected serum chemistry tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). (See 'Laboratory findings' above.) 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Correlation of interleukin-6 production and disease activity in polymyalgia rheumatica and giant cell arteritis. Arthritis Rheum 1993; 36:1286. 101. Weyand CM, Fulbright JW, Hunder GG, et al. Treatment of giant cell arteritis: interleukin-6 as a biologic marker of disease activity. Arthritis Rheum 2000; 43:1041. Topic 8225 Version 36.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 23/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate GRAPHICS Giant cell arteritis: Clinical findings in 100 patients Finding Number Sex (female/male) 69/31 Duration of manifestations before diagnosis 7* Onset (gradual/sudden) 64/36 Weight loss or anorexia 50 Malaise, fatigue, or weakness 40 Fever 42 Polymyalgia rheumatica 39 Other musculoskeletal pains 30 Synovitis 15 Sore throat 9 Symptoms related to arteries 83 Headache 68 Visual symptoms Transient 16 Fixed 14 Jaw claudication 45 Swallowing claudication or dysphagia 8 Tongue claudication 6 Limb claudication 4 Signs related to arteries 66 Artery tenderness 27 Decreased temporal artery pulsations 46 Erythematous, nodular, or swollen scalp arteries 23 Large artery bruits 21 Decreased large artery pulses 7 Visual loss 14 Ophthalmoscopic abnormalities 18 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 24/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Extraocular muscle weakness 2 Raynaud phenomenon 3 Central nervous system abnormalities 15 Range 1 to 48 months before diagnosis. Adapted from: Calamia, KT, Hunder, GG. Clin Rheum Dis 1980; 6:389. Graphic 54634 Version 3.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 25/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Aortitis in GCA on MR angiography (A) Parasagittal MR angiography image of the aorta demonstrates diffuse thickening and peripheral enhancement of the aortic wall along the aortic arch, the descending aorta, and the proximal abdominal aorta in a patient with biopsy-proven GCA. https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 26/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate (B) High-resolution, axial-delayed MR angiography image of the aorta shows thickening and enhancement of the aortic wall. GCA: giant cell arteritis; MR: magnetic resonance. Courtesy of William Docken, MD. Graphic 101604 Version 5.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 27/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate MRA of the aorta and great vessels in GCA Stenosis of the proximal and distal right subclavian artertery in a patient with large vessel GCA. The right axil artery is completely occluded; the right brachial artery is reconstituted via collateral channels. The distal left subclavian artery is severely stenosed. https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 28/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate MRA: magnetic resonance angiogram; GCA: giant cell arteritis. Courtesy of William Docken, MD. Graphic 101605 Version 4.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 29/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate PET CT scan of GCA patient with aortic involvement PET CT scan of a patient with biopsy-proven GCA, showing increased FDG uptake in the walls of the ascending and descending aorta. PET: positron emission tomography; CT: computed tomography; GCA: giant cell arteritis; FDG: fluorodeoxyglucose. From: Docken WP. Two in ammatory conditions polymyalgia rheumatica and giant cell arteritis-share clinical connection. The Rheumatologist 2013. http://www.the- rheumatologist.org/article/two-in ammatory-conditionspolymyalgia-rheumatica-and- giant-cell-arteritisshare-clinical-connection/. Copyright 2013 American College of Rheumatology. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (http://onlinelibrary.wiley.com). Graphic 100635 Version 3.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 30/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Narrowing of the left subclavian artery in giant cell arteritis Angiogram of the left subclavian artery in GCA, showing several areas of severe vascular narrowing but a proliferation of small blood vessels forming excellent collateral circulation. The long, smooth arterial tapering in large vessel GCA is often not amenable to revascularization interventions such as angioplasty or stent placement. Those types of interventions are rarely necessary. GCA: giant cell arteritis. Courtesy of John H Stone, MD, MPH. Graphic 73553 Version 8.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 31/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate External carotid artery anatomy External carotid artery and its branches in the lateral view. Reproduced with permission from: U acker R. Atlas Of Vascular Anatomy: An Angiographic Approach, Second Edition. Philadelphia: Lippincott Williams & Wilkins, 2007. Copyright 2007 Lippincott Williams & Wilkins. Graphic 50495 Version 1.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 32/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Course of the superficial temporal artery Graphic 82875 Version 2.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 33/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Enlarged temporal artery Visibly enlarged temporal artery in a patient with GCA (arrows). GCA: giant cell arteritis. Courtesy of Gene G Hunder, MD. Graphic 77618 Version 4.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 34/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Ischemic optic neuropathy Ischemic optic neuropathy in a patient with giant cell artertitis who lost vision abruptly four days prior to this examination. The optic disc is swollen and its margins are blurred. Courtesy of Gene Hunder, MD. Graphic 70141 Version 1.0 https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 35/36 7/7/23, 11:15 AM Clinical manifestations of giant cell arteritis - UpToDate Contributor Disclosures Carlo Salvarani, MD No relevant financial relationship(s) with ineligible companies to disclose. Francesco Muratore, MD No relevant financial relationship(s) with ineligible companies to disclose. Kenneth J Warrington, MD Grant/Research/Clinical Trial Support: Eli Lilly [Giant cell arteritis]; GSK [Giant cell arteritis]; Kiniksa [Giant cell arteritis]. Other Financial Interest: Chemocentryx [Honoraria ANCA-associated vasculitis]. All of the relevant financial relationships listed have been mitigated. Jonathan Trobe, MD No relevant financial relationship(s) with ineligible companies to disclose. Philip Seo, MD, MHS No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-arteritis/print 36/36