Giant Cell Arteritis

Abstract

Giant cell arteritis (GCA) is the most common primary vasculitis and is associated with potential bilateral blindness. Neither clinical nor laboratory evidence is simple and unequivocal for this disease, which usually requires rapid and reliable diagnosis and therapy. The ophthalmologist should consider GCA with the following ocular symptoms: visual loss or visual field defects, transient visual disturbances (amaurosis fugax), diplopia, eye pain, or new onset head or jaw claudication. An immediate ophthalmological examination with slit lamp, ophthalmoscopy, and visual field, as well as color duplex ultrasound of the temporal artery should be performed. If there is sufficient clinical suspicion of GCA, corticosteroid therapy should be initiated immediately, with prompt referral to a rheumatologist/internist and, if necessary, temporal artery biopsy should be arranged. Numerous developments in modern imaging with colour duplex ultrasonography, MRI, and PET-CT have the potential to compete with the classical, well-established biopsy of a temporal artery. Early determination of ESR and CRP may support RZA diagnosis. Therapeutically, steroid-sparing immunosuppression with IL-6 blockade or methotrexate can be considered. These developments have led to a revision of both the classification criteria and the diagnostic and therapeutic recommendations of the American College of Rheumatologists and the European League against Rheumatism, which are summarised here for ophthalmology.

Keywords

giant cell arteritis - AION - duplex ultrasound - EULAR - MRI

• References/Literatur

• 1 Li KJ, Semenov D, Turk M. et al. A meta-analysis of the epidemiology of giant cell arteritis across time and space. Arthritis Res Ther 2021; 23: 82
• 2 Mukhtyar C, Guillevin L, Cid MC. et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009; 68: 318-323
• 3 Hellmich B, Agueda A, Monti S. et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2020; 79: 19-30
• 4 Monti S, Águeda AF, Luqmani RA. et al. Systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis: focus on giant cell arteritis. RMD Open 2019; 5: e001003
• 5 Borchers AT, Gershwin ME. Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev 2012; 11: A544-A554
• 6 Chandran AK, Udayakumar PD, Crowson CS. et al. The incidence of giant cell arteritis in Olmsted County, Minnesota, over a 60-year period 1950–2009. Scand J Rheumatol 2015; 44: 215-218
• 7 Bilton EJ, Mollan SP. Giant cell arteritis: reviewing the advancing diagnostics and management. Eye (Lond) 2023; 37: 2365-2373
• 8 Weyand CM, Hicok KC, Hunder GG. et al. The HLA-DRB1 locus as a genetic component in giant cell arteritis. Mapping of a disease-linked sequence motif to the antigen binding site of the HLA-DR molecule. J Clin Invest 1992; 90: 2355-2361
• 9 Carmona FD, González-Gay MA, Martín J. Genetic component of giant cell arteritis. Rheumatology (Oxford) 2014; 53: 6-18
• 10 Larsson K, Mellström D, Nordborg E. et al. Early menopause, low body mass index, and smoking are independent risk factors for developing giant cell arteritis. Ann Rheum Dis 2006; 65: 529-532
• 11 Li L, Neogi T, Jick S. Giant cell arteritis and vascular disease-risk factors and outcomes: a cohort study using UK Clinical Practice Research Datalink. Rheumatology (Oxford) 2017; 56: 753-762
• 12 Mollan SP, Paemeleire K, Versijpt J. et al. European Headache Federation recommendations for neurologists managing giant cell arteritis. J Headache Pain 2020; 21: 28
• 13 Neß T, Schmidt W. [Eye involvement in large vessel vasculitis (giant cell arteritis and Takayasuʼs arteritis)]. Ophthalmologe 2019; 116: 899-914
• 14 Ness T, Auw-Hädrich C, Schmidt D. [Temporal arteritis (giant cell arteritis). Clinical picture, histology, and treatment]. Ophthalmologe 2006; 103: 296-301
• 15 Salvarani C, Cimino L, Macchioni P. et al. Risk factors for visual loss in an Italian population-based cohort of patients with giant cell arteritis. Arthritis Rheum 2005; 53: 293-297
• 16 Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol 1998; 125: 509-520
• 17 Danesh-Meyer H, Savino PJ, Gamble GG. Poor prognosis of visual outcome after visual loss from giant cell arteritis. Ophthalmology 2005; 112: 1098-1103
• 18 Hayreh SS, Zimmerman B. Visual deterioration in giant cell arteritis patients while on high doses of corticosteroid therapy. Ophthalmology 2003; 110: 1204-1215
• 19 Curumthaullee MF, Liozon E, Dumonteil S. et al. Features and risk factors for new (secondary) permanent visual involvement in giant cell arteritis. Clin Exp Rheumatol 2022; 40: 734-740
• 20 Dejaco C, Duftner C, Buttgereit F. et al. The spectrum of giant cell arteritis and polymyalgia rheumatica: revisiting the concept of the disease. Rheumatology (Oxford) 2017; 56: 506-515
• 21 de Boysson H, Liozon E, Espitia O. et al. Different patterns and specific outcomes of large-vessel involvements in giant cell arteritis. J Autoimmun 2019; 103: 102283
• 22 Kaushik M, Ponte C, Mollan SP. Current advances in giant cell arteritis. Curr Opin Neurol 2021; 34: 133-141
• 23 Calamia KT, Hunder GG. Giant cell arteritis (temporal arteritis) presenting as fever of undetermined origin. Arthritis Rheum 1981; 24: 1414-1418
• 24 Hunder GG. Clinical features of GCA/PMR. Clin Exp Rheumatol 2000; 18: S6-8
• 25 Walvick MD, Walvick MP. Giant cell arteritis: laboratory predictors of a positive temporal artery biopsy. Ophthalmology 2011; 118: 1201-1204
• 26 van der Geest KSM, Sandovici M, Brouwer E. et al. Diagnostic Accuracy of Symptoms, Physical Signs, and Laboratory Tests for Giant Cell Arteritis: A Systematic Review and Meta-analysis. JAMA Intern Med 2020; 180: 1295-1304
• 27 Gonzalez-Gay MA, Lopez-Diaz MJ, Barros S. et al. Giant cell arteritis: laboratory tests at the time of diagnosis in a series of 240 patients. Medicine (Baltimore) 2005; 84: 277-290
• 28 Kermani TA, Schmidt J, Crowson CS. et al. Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of giant cell arteritis. Semin Arthritis Rheum 2012; 41: 866-871
• 29 Parikh M, Miller NR, Lee AG. et al. Prevalence of a normal C-reactive protein with an elevated erythrocyte sedimentation rate in biopsy-proven giant cell arteritis. Ophthalmology 2006; 113: 1842-1845
• 30 Laria A, Zoli A, Bocci M. et al. Systematic review of the literature and a case report informing biopsy-proven giant cell arteritis (GCA) with normal C-reactive protein. Clin Rheumatol 2012; 31: 1389-1393
• 31 Lyons HS, Quick V, Sinclair AJ. et al. A new era for giant cell arteritis. Eye (Lond) 2020; 34: 1013-1026
• 32 Luqmani R, Lee E, Singh S. et al. The Role of Ultrasound Compared to Biopsy of Temporal Arteries in the Diagnosis and Treatment of Giant Cell Arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study. Health Technol Assess 2016; 20: 1-238
• 33 Rubenstein E, Maldini C, Gonzalez-Chiappe S. et al. Sensitivity of temporal artery biopsy in the diagnosis of giant cell arteritis: a systematic literature review and meta-analysis. Rheumatology (Oxford) 2020; 59: 1011-1020
• 34 Poller DN, van Wyk Q, Jeffrey MJ. The importance of skip lesions in temporal arteritis. J Clin Pathol 2000; 53: 137-139
• 35 Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum 1990; 33: 1074-1087
• 36 Ness T, Bley TA, Schmidt WA. et al. The diagnosis and treatment of giant cell arteritis. Dtsch Arztebl Int 2013; 110: 376-385
• 37 Maleszewski JJ, Younge BR, Fritzlen JT. et al. Clinical and pathological evolution of giant cell arteritis: a prospective study of follow-up temporal artery biopsies in 40 treated patients. Mod Pathol 2017; 30: 788-796
• 38 Dejaco C, Ramiro S, Bond M. et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update. Ann Rheum Dis 2023;
• 39 Schmidt WA. Ultrasound in the diagnosis and management of giant cell arteritis. Rheumatology (Oxford) 2018; 57 (Suppl. 2) ii22-ii31
• 40 Casteleyn V, Schmidt WA. [Imaging of large vessel vasculitis]. Z Rheumatol 2023; 82: 646-653
• 41 Rinagel M, Chatelus E, Jousse-Joulin S. et al. Diagnostic performance of temporal artery ultrasound for the diagnosis of giant cell arteritis: a systematic review and meta-analysis of the literature. Autoimmun Rev 2019; 18: 56-61
• 42 van der Geest KSM, Borg F, Kayani A. et al. Novel ultrasonographic Halo Score for giant cell arteritis: assessment of diagnostic accuracy and association with ocular ischaemia. Ann Rheum Dis 2020; 79: 393-399
• 43 Bley TA, Wieben O, Vaith P. et al. Magnetic resonance imaging depicts mural inflammation of the temporal artery in giant cell arteritis. Arthritis Rheum 2004; 51: 1062-1063 author reply 1064
• 44 Bley TA, Uhl M, Carew J. et al. Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol 2007; 28: 1722-1727
• 45 Bley TA, Weiben O, Uhl M. et al. Assessment of the cranial involvement pattern of giant cell arteritis with 3 T magnetic resonance imaging. Arthritis Rheum 2005; 52: 2470-2477
• 46 Hauenstein C, Reinhard M, Geiger J. et al. Effects of early corticosteroid treatment on magnetic resonance imaging and ultrasonography findings in giant cell arteritis. Rheumatology (Oxford) 2012; 51: 1999-2003
• 47 Bley TA, Ness T, Warnatz K. et al. Influence of corticosteroid treatment on MRI findings in giant cell arteritis. Clin Rheumatol 2007; 26: 1541-1543
• 48 Sammel AM, Hsiao E, Schembri G. et al. Diagnostic Accuracy of Positron Emission Tomography/Computed Tomography of the Head, Neck, and Chest for Giant Cell Arteritis: A Prospective, Double-Blind, Cross-Sectional Study. Arthritis Rheumatol 2019; 71: 1319-1328
• 49 Nielsen BD, Gormsen LC. 18F-Fluorodeoxyglucose PET/Computed Tomography in the Diagnosis and Monitoring of Giant Cell Arteritis. PET Clin 2020; 15: 135-145
• 50 Nielsen BD, Gormsen LC, Hansen IT. et al. Three days of high-dose glucocorticoid treatment attenuates large-vessel 18F-FDG uptake in large-vessel giant cell arteritis but with a limited impact on diagnostic accuracy. Eur J Nucl Med Mol Imaging 2018; 45: 1119-1128
• 51 Ponte C, Grayson PC, Robson JC. et al. 2022 American College of Rheumatology/EULAR classification criteria for giant cell arteritis. Ann Rheum Dis 2022; 81: 1647-1653
• 52 Mazlumzadeh M, Hunder GG, Easley KA. et al. Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo-controlled, randomized prospective clinical trial. Arthritis Rheum 2006; 54: 3310-3318
• 53 Cornblath WT, Eggenberger ER. Progressive visual loss from giant cell arteritis despite high-dose intravenous methylprednisolone. Ophthalmology 1997; 104: 854-858
• 54 Stone JH, Spotswood H, Unizony SH. et al. New-onset versus relapsing giant cell arteritis treated with tocilizumab: 3-year results from a randomized controlled trial and extension. Rheumatology (Oxford) 2022; 61: 2915-2922
• 55 Stone JH, Tuckwell K, Dimonaco S. et al. Trial of Tocilizumab in Giant-Cell Arteritis. N Engl J Med 2017; 377: 317-328
• 56 Matza MA, Dagincourt N, Mohan SV. et al. Outcomes during and after long-term tocilizumab treatment in patients with giant cell arteritis. RMD Open 2023; 9: e002923
• 57 Unizony S, McCulley TJ, Spiera R. et al. Clinical outcomes of patients with giant cell arteritis treated with tocilizumab in real-world clinical practice: decreased incidence of new visual manifestations. Arthritis Res Ther 2021; 23: 8
• 58 Mackie SL, Dejaco C, Appenzeller S. et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis: executive summary. Rheumatology (Oxford) 2020; 59: 487-494
• 59 Mahr AD, Jover JA, Spiera RF. et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum 2007; 56: 2789-2797
• 60 Seror R, Baron G, Hachulla E. et al. Adalimumab for steroid sparing in patients with giant-cell arteritis: results of a multicentre randomised controlled trial. Ann Rheum Dis 2014; 73: 2074-2081
• 61 Adizie T, Christidis D, Dharmapaliah C. et al. Efficacy and tolerability of leflunomide in difficult-to-treat polymyalgia rheumatica and giant cell arteritis: a case series. Int J Clin Pract 2012; 66: 906-909
• 62 Diamantopoulos AP, Hetland H, Myklebust G. Leflunomide as a corticosteroid-sparing agent in giant cell arteritis and polymyalgia rheumatica: a case series. Biomed Res Int 2013; 2013: 120638
• 63 Fragoulis GE, Nikiphorou E, Dey M. et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis 2023; 82: 742-753