Treatment of giant cell (temporal) arteritis
- Gene G Hunder, MD
Gene G Hunder, MD
- Section Editor — Vasculitis
- Emeritus Consultant
- Professor Emeritus
- Mayo Clinic College of Medicine
- Section Editors
- Jonathan Trobe, MD
Jonathan Trobe, MD
- Section Editor — Ophthalmology
- Professor of Ophthalmology and Visual Sciences
- Professor of Neurology
- University of Michigan Kellogg Eye Center
- Eric L Matteson, MD, MPH
Eric L Matteson, MD, MPH
- Section Editor — Treatment Issues in Rheumatology
- Division of Rheumatology
- Professor of Medicine
- Mayo Clinic College of Medicine
Giant cell arteritis (GCA), also known as temporal arteritis, is a chronic vasculitis of medium and large vessels that occurs only in individuals older than 50 years of age . The mean age at diagnosis in patients with GCA is 72. Many of the most concerning features of the disease (eg, anterior ischemic optic neuropathy resulting in visual loss) result from vascular inflammation involving cranial branches of the arteries that originate from the aortic arch [2,3]. The disease is generalized, however, and may also involve the aorta itself, leading to aneurysms of the thoracic and abdominal aorta and also to ischemic symptoms (claudication) in the extremities.
The treatment and prognosis of giant cell arteritis (GCA) are reviewed here. The clinical manifestations and diagnosis of this disorder are discussed separately. (See "Clinical manifestations of giant cell (temporal) arteritis" and "Diagnosis of giant cell (temporal) arteritis".)
Glucocorticoid treatment should be instituted promptly once the diagnosis of GCA is suspected strongly, often even before it is confirmed. For patients in whom the diagnostic suspicion of GCA is high, especially those with recent or threatened vascular complications such as visual loss, therapy should be started immediately. A temporal artery biopsy should be obtained as soon as possible, but treatment should not be withheld while awaiting the performance or the results of the biopsy. (See 'Visual loss at diagnosis' below and "Diagnosis of giant cell (temporal) arteritis".)
If the temporal or other artery biopsies reveal no evidence of arteritis but if clinical suspicion of GCA remains strong, glucocorticoid treatment should be continued . Even with optimal bilateral temporal artery biopsy performance, false-negative results occur in at least 9 percent of GCA cases and probably in even higher percentages in most settings . The therapeutic regimen for GCA is somewhat different than that for patients who are believed to have only polymyalgia rheumatica (PMR). In general, the treatment of PMR requires lower doses of glucocorticoids. (See "Diagnosis of giant cell (temporal) arteritis" and "Treatment of polymyalgia rheumatica" and 'Glucocorticoid tapering' below.)
Efficacy — Glucocorticoids have not been studied in a placebo-controlled manner in GCA. Their effectiveness in this disorder is so well-established, however, by years of use and knowledge of the consequences of untreated disease, that it would be unethical to conduct such a study at this time. Evidence for efficacy is based upon several series in which the use of glucocorticoids improved or resolved symptoms and decreased the risk of vascular complications when compared with the rates of such events in patients with the disease prior to the glucocorticoid era [5-8].
Subscribers log in hereLiterature review current through: May 2017. | This topic last updated: Sep 14, 2016.References
- Hunder GG. Giant cell arteritis and polymyalgia rheumatica. In: Textbook of Rheumatology, 5th, Kelly WN, Harris ED, Ruddy S, Sledge CB (Eds), WB Saunders, Philadelphia 1996.
- Evans JM, Bowles CA, Bjornsson J, et al. Thoracic aortic aneurysm and rupture in giant cell arteritis. A descriptive study of 41 cases. Arthritis Rheum 1994; 37:1539.
- Klein RG, Hunder GG, Stanson AW, Sheps SG. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975; 83:806.
- Hall S, Persellin S, Lie JT, et al. The therapeutic impact of temporal artery biopsy. Lancet 1983; 2:1217.
- Salvarani C, Macchioni PL, Tartoni PL, et al. Polymyalgia rheumatica and giant cell arteritis: a 5-year epidemiologic and clinical study in Reggio Emilia, Italy. Clin Exp Rheumatol 1987; 5:205.
- Delecoeuillerie G, Joly P, Cohen de Lara A, Paolaggi JB. Polymyalgia rheumatica and temporal arteritis: a retrospective analysis of prognostic features and different corticosteroid regimens (11 year survey of 210 patients). Ann Rheum Dis 1988; 47:733.
- Lundberg I, Hedfors E. Restricted dose and duration of corticosteroid treatment in patients with polymyalgia rheumatica and temporal arteritis. J Rheumatol 1990; 17:1340.
- Kyle V, Hazleman BL. Treatment of polymyalgia rheumatica and giant cell arteritis. II. Relation between steroid dose and steroid associated side effects. Ann Rheum Dis 1989; 48:662.
- Hunder GG, Sheps SG, Allen GL, Joyce JW. Daily and alternate-day corticosteroid regimens in treatment of giant cell arteritis: comparison in a prospective study. Ann Intern Med 1975; 82:613.
- Myles AB, Perera T, Ridley MG. Prevention of blindness in giant cell arteritis by corticosteroid treatment. Br J Rheumatol 1992; 31:103.
- 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.
- Hayreh SS, Zimmerman B, Kardon RH. Visual improvement with corticosteroid therapy in giant cell arteritis. Report of a large study and review of literature. Acta Ophthalmol Scand 2002; 80:355.
- Durand M, Thomas SL. Incidence of infections in patients with giant cell arteritis: a cohort study. Arthritis Care Res (Hoboken) 2012; 64:581.
- Foroozan R, Deramo VA, Buono LM, et al. Recovery of visual function in patients with biopsy-proven giant cell arteritis. Ophthalmology 2003; 110:539.
- Roche NE, Fulbright JW, Wagner AD, et al. Correlation of interleukin-6 production and disease activity in polymyalgia rheumatica and giant cell arteritis. Arthritis Rheum 1993; 36:1286.
- Macchioni P, Boiardi L, Meliconi R, et al. Elevated soluble intercellular adhesion molecule 1 in the serum of patients with polymyalgia rheumatica: influence of steroid treatment. J Rheumatol 1994; 21:1860.
- 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.
- Kermani TA, Warrington KJ, Cuthbertson D, et al. Disease Relapses among Patients with Giant Cell Arteritis: A Prospective, Longitudinal Cohort Study. J Rheumatol 2015; 42:1213.
- Weyand CM, Kaiser M, Yang H, et al. Therapeutic effects of acetylsalicylic acid in giant cell arteritis. Arthritis Rheum 2002; 46:457.
- Liozon E, Herrmann F, Ly K, et al. Risk factors for visual loss in giant cell (temporal) arteritis: a prospective study of 174 patients. Am J Med 2001; 111:211.
- Nesher G, Berkun Y, Mates M, et al. Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum 2004; 50:1332.
- Lee MS, Smith SD, Galor A, Hoffman GS. Antiplatelet and anticoagulant therapy in patients with giant cell arteritis. Arthritis Rheum 2006; 54:3306.
- García-Martínez A, Hernández-Rodríguez J, Arguis P, et al. Development of aortic aneurysm/dilatation during the followup of patients with giant cell arteritis: a cross-sectional screening of fifty-four prospectively followed patients. Arthritis Rheum 2008; 59:422.
- Kermani TA, Warrington KJ, Crowson CS, et al. Large-vessel involvement in giant cell arteritis: a population-based cohort study of the incidence-trends and prognosis. Ann Rheum Dis 2013; 72:1989.
- Bossert M, Prati C, Balblanc JC, et al. Aortic involvement in giant cell arteritis: current data. Joint Bone Spine 2011; 78:246.
- Evans JM, O'Fallon WM, Hunder GG. Increased incidence of aortic aneurysm and dissection in giant cell (temporal) arteritis. A population-based study. Ann Intern Med 1995; 122:502.
- García-Martínez A, Arguis P, Prieto-González S, et al. Prospective long term follow-up of a cohort of patients with giant cell arteritis screened for aortic structural damage (aneurysm or dilatation). Ann Rheum Dis 2014; 73:1826.
- Hoffman GS, Cid MC, Hellmann DB, et al. A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum 2002; 46:1309.
- Jover JA, Hernández-García C, Morado IC, et al. Combined treatment of giant-cell arteritis with methotrexate and prednisone. a randomized, double-blind, placebo-controlled trial. Ann Intern Med 2001; 134:106.
- Spiera RF, Mitnick HJ, Kupersmith M, et al. A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis (GCA). Clin Exp Rheumatol 2001; 19:495.
- 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.
- Villiger PM, Adler S, Kuchen S, et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet 2016; 387:1921.
- Unizony S, Arias-Urdaneta L, Miloslavsky E, et al. Tocilizumab for the treatment of large-vessel vasculitis (giant cell arteritis, Takayasu arteritis) and polymyalgia rheumatica. Arthritis Care Res (Hoboken) 2012; 64:1720.
- Seitz M, Reichenbach S, Bonel HM, et al. Rapid induction of remission in large vessel vasculitis by IL-6 blockade. A case series. Swiss Med Wkly 2011; 141:w13156.
- Beyer C, Axmann R, Sahinbegovic E, et al. Anti-interleukin 6 receptor therapy as rescue treatment for giant cell arteritis. Ann Rheum Dis 2011; 70:1874.
- Salvarani C, Magnani L, Catanoso M, et al. Tocilizumab: a novel therapy for patients with large-vessel vasculitis. Rheumatology (Oxford) 2012; 51:151.
- Sciascia S, Rossi D, Roccatello D. Interleukin 6 blockade as steroid-sparing treatment for 2 patients with giant cell arteritis. J Rheumatol 2011; 38:2080.
- Loricera J, Blanco R, Hernández JL, et al. Tocilizumab in giant cell arteritis: Multicenter open-label study of 22 patients. Semin Arthritis Rheum 2015; 44:717.
- Régent A, Redeker S, Deroux A, et al. Tocilizumab in Giant Cell Arteritis: A Multicenter Retrospective Study of 34 Patients. J Rheumatol 2016; 43:1547.
- Quartuccio L, Maset M, De Maglio G, et al. Role of oral cyclophosphamide in the treatment of giant cell arteritis. Rheumatology (Oxford) 2012; 51:1677.
- Henes JC, Mueller M, Pfannenberg C, et al. Cyclophosphamide for large vessel vasculitis: assessment of response by PET/CT. Clin Exp Rheumatol 2011; 29:S43.
- de Boysson H, Boutemy J, Creveuil C, et al. Is there a place for cyclophosphamide in the treatment of giant-cell arteritis? A case series and systematic review. Semin Arthritis Rheum 2013; 43:105.
- Hoffman GS, Cid MC, Rendt-Zagar KE, et al. Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial. Ann Intern Med 2007; 146:621.
- 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.
- Martínez-Taboada VM, Rodríguez-Valverde V, Carreño L, et al. A double-blind placebo controlled trial of etanercept in patients with giant cell arteritis and corticosteroid side effects. Ann Rheum Dis 2008; 67:625.
- Do DD, Jeanneret C, Mahler F. Images in vascular medicine. Giant cell arteritis of axillary artery. Vasc Med 1996; 1:293.
- Monte R, González-Gay MA, García-Porrúa C, et al. Successful response to angioplasty in a patient with upper limb ischaemia secondary to giant cell arteritis. Br J Rheumatol 1998; 37:344.
- Dellaripa PF, Eisenhauer AC. Bilateral percutaneous balloon angioplasty of the axillary arteries in a patient with giant cell arteritis and upper extremity ischemic symptoms not responsive to corticosteroids. J Rheumatol 1998; 25:1429.
- Amann-Vesti BR, Koppensteiner R, Rainoni L, et al. Immediate and long-term outcome of upper extremity balloon angioplasty in giant cell arteritis. J Endovasc Ther 2003; 10:371.
- Both M, Aries PM, Müller-Hülsbeck S, et al. Balloon angioplasty of arteries of the upper extremities in patients with extracranial giant-cell arteritis. Ann Rheum Dis 2006; 65:1124.
- Matteson EL, Gold KN, Bloch DA, Hunder GG. Long-term survival of patients with giant cell arteritis in the American College of Rheumatology giant cell arteritis classification criteria cohort. Am J Med 1996; 100:193.
- Aiello PD, Trautmann JC, McPhee TJ, et al. Visual prognosis in giant cell arteritis. Ophthalmology 1993; 100:550.
- Liozon E, Loustaud-Ratti V, Ly K, et al. Visual prognosis in extremely old patients with temporal (giant cell) arteritis. J Am Geriatr Soc 2003; 51:722.
- 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.
- Cid MC, Font C, Oristrell J, et al. Association between strong inflammatory response and low risk of developing visual loss and other cranial ischemic complications in giant cell (temporal) arteritis. Arthritis Rheum 1998; 41:26.
- Lopez-Diaz MJ, Llorca J, Gonzalez-Juanatey C, et al. The erythrocyte sedimentation rate is associated with the development of visual complications in biopsy-proven giant cell arteritis. Semin Arthritis Rheum 2008; 38:116.
- Tomasson G, Peloquin C, Mohammad A, et al. Risk for cardiovascular disease early and late after a diagnosis of giant-cell arteritis: a cohort study. Ann Intern Med 2014; 160:73.
- TREATMENT APPROACH
- INITIAL TREATMENT
- - Efficacy
- - Initial dose
- - Visual loss at diagnosis
- - Polymyalgia rheumatica
- - Adverse effects
- - Glucocorticoid tapering
- Monitoring and gauging disease activity
- Antiplatelet therapy
- Other measures
- RESISTANT DISEASE AND GLUCOCORTICOID-SPARING AGENTS
- Anti-TNF therapy
- IS REVASCULARIZATION INDICATED?
- Visual loss
- Cardiovascular disease
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS