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Giant Cell Arteritis and Polymyalgia Rheumatica

  • James R. Stone
  • Misha Pless
  • Carlo Salvarani
  • Nicolo Pipitone
  • Simmons Lessell
  • John H. Stone

Abstract

Giant cell arteritis (GCA) is a form a large- to medium-vessel vasculitis that involves the aorta and its branches. Polymyalgia rheumatica (PMR), a syndrome of muscle pain and stiffness in the neck, shoulders, and hips, often occurs with GCA but can occur independently. GCA predominantly affects the second- to fifth-order aortic branches, often in the extracranial arteries of the head. The mean age at diagnosis of GCA is approximately 72. The diagnosis of GCA is made usually by biopsy of the temporal artery. GCA associated with granulomatous inflammation within the blood vessel wall, but granulomatous changes are not always evident early in the disease. Giant cells occur in a slight majority of cases of positive temporal artery biopsies. Estimates of the frequency of giant cells in temporal artery biopsy tissues are on the order of 50–80%. In GCA, clinical symptoms of local vascular inflam-mation and vascular insufficiency are usually accompanied or preceded by a systemic inflammatory process. Visual loss is the most feared complication of GCA. Visual loss may occur through the syndrome of anterior ischemic optic neuropathy, caused by narrowing of the posterior ciliary artery, as well as through other arterial insufficiency syndromes. Glucocorticoids are the cornerstone of treatment for GCA and PMR. PMR occurring in the absence of GCA requires a lower dose of prednisone for disease control.

Keywords

Giant Cell Arteritis Temporal Artery Temporal Arteritis Temporal Artery Biopsy Nonarteritic Anterior Ischemic Optic Neuropathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. Achkar A, Lie J, Hunder G, et al How does previous corticosteroid treatment affect the biopsy findings in giant cell (temporal) arteritis? Ann Intern Med 1994;120:987–92PubMedCrossRefGoogle Scholar
  2. Allsop CJ, Gallagher PJ. Temporal artery biopsy in giant-cell arteritis: a reappraisal. Am J Surg Pathol 1981;5:317–23PubMedCrossRefGoogle Scholar
  3. Amor-Dorado JC, Llorca J, Garcia-Porrua C, et al Audiovestibular manifestations in giant cell arteritis: a prospective study. Medicine (Baltimore) 2003;82(1):13–26CrossRefGoogle Scholar
  4. Andersson R, Malmvall BE, Bengtsson BA. Long-term corticosteroid treatment in giant cell arteritis. Acta Med Scand 1986;220(5):465–9PubMedCrossRefGoogle Scholar
  5. Bley TA, Brink I, Reinhard M. [Imaging procedures for giant cell arteri-tis (Horton's disease)]. Ophthalmologe 2006;103(4):308–16PubMedCrossRefGoogle Scholar
  6. 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 2005a;52(8):2470–7CrossRefGoogle Scholar
  7. Bley TA, Wieben O, Uhl M, et al High-resolution MRI in giant cell arteritis: imaging of the wall of the superficial temporal artery. AJR Am J Roentgenol 2005b;184(1):283–7CrossRefGoogle Scholar
  8. Blockmans D. The use of (18F)fluoro-deoxyglucose positron emission tomography in the assessment of large vessel vasculitis. Clin Exp Rheumatol 2003;21(6 Suppl 32):S15–22PubMedGoogle Scholar
  9. Blockmans D. PET in large-vessel vasculitis and polymyalgia rheu-matica. In: Fourth International Conference on Giant Cell Arteritis and Polymyalgia Rheumatica, New York; 2008Google Scholar
  10. Blockmans D, De Ceuninck L, Vanderschueren S, et al Repetitive 18F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a prospective study of 35 patients. Arthritis Rheum 2006;55 (1):131–7PubMedCrossRefGoogle Scholar
  11. Bongartz T, Matteson EL. Large-vessel involvement in giant cell arteri-tis. Curr Opin Rheumatol 2006;18(1):10–7PubMedCrossRefGoogle Scholar
  12. Boyev LR, Miller NR, Green WR. Efficacy of unilateral versus bilateral temporal artery biopsies for the diagnosis of giant cell arteritis. Am J Ophthalmol 1999;128:211–5PubMedCrossRefGoogle Scholar
  13. Brack A, Martinez-Taboada V, Stanson A, Goronzy JJ, Weyand CM. Disease pattern in cranial and large-vessel giant cell arteritis. Arthritis Rheum 1999;42(2):311–7PubMedCrossRefGoogle Scholar
  14. Brass SD, Durand ML, Stone JH, et al A 59 year-old man with chronic daily headaches. N Engl J Med 2008; 359(21):2267–78.PubMedCrossRefGoogle Scholar
  15. Caselli RJ, Daube JR, Hunder GG, et al Peripheral neuropathic syndromes in giant cell (temporal) arteritis. Neurology 1988;38: 685–9PubMedCrossRefGoogle Scholar
  16. Caselli RJ, Hunder GG, Whisnant JP. Neurologic disease in biopsy-proven giant cell (temporal) arteritis. Neurology 1988; 38:352–9.PubMedCrossRefGoogle Scholar
  17. Churchill CH, Abril A, Krishna M, et al Jaw claudication in primary amyloidosis: unusual presentation of a rare disease. J Rheumatol 2003;30:2283–6PubMedGoogle Scholar
  18. Cohen MD, Ginsburg WW, Allen GL. Facial swelling in giant cell arteritis. J Rheumatol 1982;9:325–7PubMedGoogle Scholar
  19. Duhaut P, Berruyer M, Pinede L, et al Anticardiolipin antibodies and giant cell arteritis: a prospective, multicenter case-control study. Groupe de Recherche sur l'Arterite a Cellules Geantes. Arthritis Rheum 1998;41(4):701–9PubMedCrossRefGoogle Scholar
  20. Esposito AL, Gleckman RA. Fever of unknown origin in the elderly. J Am Geriatr Soc 1978;26:498–505PubMedGoogle Scholar
  21. Esteban MJ, Font C, Hernandex-Rodriguez J, et al Small-vessel vascu-litis surrounding a spared temporal artery: clinical and pathological findings in a series of twenty-eight patients. Arthritis Rheum 2001;44:1387–95PubMedCrossRefGoogle Scholar
  22. Evans J, O'Fallon W, Hunder G. Increased incidence of aortic aneu-rysm and dissection in giant cell (temporal) arteritis. Ann Int Med 1995;122:502–7PubMedCrossRefGoogle Scholar
  23. Font RL, Prabhakaran VC. Histological parameters helpful in recognizing steroid-treated temporal arteritis: an analysis of 35 cases. Br J Ophthalmol 2007;91:204–9PubMedCrossRefGoogle Scholar
  24. Friedman G, Friedman B. The sensation of facial swelling in temporal arteritis: a predictor for the development of visual disturbance. Postgrad Med J 1986;62(733):1019–20PubMedCrossRefGoogle Scholar
  25. Genereau T, Lortholary O, Pottier M, et al Temporal artery biopsy. A diagnostic tool for systemic necrotizing vasculitis. Arthritis Rheum 1999;42:2674–81PubMedCrossRefGoogle Scholar
  26. Gertz MA, Kyle RA, Griffing WL, et al Jaw claudication in primary systemic amyloidosis. Medicine 1986;65:173–9PubMedCrossRefGoogle Scholar
  27. Gooi P, Brownstein S, Rawlings N. Temporal arteritis: a dilemma in clinical and pathological diagnosis. Can J Ophthalmol 2008;43: 119–20PubMedCrossRefGoogle Scholar
  28. Gordon L, Levin L. Visual loss in giant cell arteritis. JAMA 1998; 280: 385–6PubMedCrossRefGoogle Scholar
  29. Graham E, Holland A, Avery A, Russell RW. Prognosis in giant-cell arteritis. Br Med J (Clin Res Ed) 1981;282(6260):269–71CrossRefGoogle Scholar
  30. Hall S, Lie J, Kurland L, et al The therapeutic impact of temporal artery biopsy. Lancet 1983;2:1217–20PubMedCrossRefGoogle Scholar
  31. Hausch RC, Harrington T. Temporal arteritis and sensorineural hearing loss. Semin Arthritis Rheum 1998;28(3):206–9PubMedCrossRefGoogle Scholar
  32. Hayreh SS, Podhajsky PA, Zimmerman B. Occult giant cell arteritis: ocular manifestations. Am J Ophthalmol 1998a;125(4):521–6CrossRefGoogle Scholar
  33. Hayreh SS, Podhajsky PA, Zimmerman B. Ocular manifestations of giant cell arteritis. Am J Ophthalmol 1998b;125(4):509–20CrossRefGoogle Scholar
  34. Hayreh SS, Zimmerman B, Kardon RH. Visual improvement with cor-ticosteroid therapy in giant cell arteritis. Report of a large study and review of literature. Acta Ophthalmol Scand 2002;80:355–67PubMedCrossRefGoogle Scholar
  35. Hellmann DB, Uhlfelder ML, Stone JH, et al Domains of quality of life important to patients with giant cell arteritis. Arthritis Rheum 2003;49(6):819–25PubMedCrossRefGoogle Scholar
  36. 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(5):1309–18PubMedCrossRefGoogle Scholar
  37. Horton BT, Magath TB, Brown GE. Undescribed forms of arteritis of temporal vessels. Proc Staff Meet Mayo Clin 1932;7:700–1Google Scholar
  38. Horton BT, Magath TB, Brown GE. Arteritis of temporal vessels: report of 7 cases. Proc Staff Meet Mayo Clin 1937;12:548–53Google Scholar
  39. 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;82:613–8PubMedCrossRefGoogle Scholar
  40. Huston KA, Hunder GG, Lie JT, et al Temporal arteritis: a 25-year epi-demiologic, clinical, and pathologic study. Ann Intern Med 1978; 88(2):162–7PubMedCrossRefGoogle Scholar
  41. Jover JA, Hernandez-Garcia 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 (2):106–14PubMedCrossRefGoogle Scholar
  42. Karahaliou M, et al Colour duplex sonography of temporal arteries before decision for biopsy: a prospective study in 55 patients with suspected giant cell arteritis. Arthritis Res Ther 2006;8:R116PubMedCrossRefGoogle Scholar
  43. Kyle V, Hazleman B. Treatment of polymyalgia rheumatica and giant cell arteritis. II. Relation between corticosteroid dose and corticos-teroid-associated diseases. Ann Rheum Dis 1989;48:662–6PubMedCrossRefGoogle Scholar
  44. Kyle V, Hazleman BL. The clinical and laboratory course of polymyal-gia rheumatica/giant cell arteritis after the first two months of treatment. Ann Rheum Dis 1993;52(12):847–50PubMedCrossRefGoogle Scholar
  45. Kyle V, Wraight EP, Hazleman BL. Liver scan abnormalities in polymyal-gia rheumatica/giant cell arteritis. Clin Rheumatol 1991;10(3):294–7PubMedCrossRefGoogle Scholar
  46. Larson T, Hall S, Hepper N, et al Respiratory tract symptoms as a clue to giant cell arteritis. Ann Intern Med 1984;101:594–7PubMedCrossRefGoogle Scholar
  47. Lee CC, Su WW, Hunder GG. Dysarthria associated with giant cell arteritis. J Rheumatol 1999;26:931–2PubMedGoogle Scholar
  48. Lee MS, Smith SD, Gabor A. Antiplatelet and anticoagulant therapy in patients with giant cell arteritis. Arthritis Rheum 2006;54:3306–9PubMedCrossRefGoogle Scholar
  49. Lessell S. Nonarteritic anterior ischemic optic neuropathy: enigma variations. Arch Ophthalmol 1999;117:386–8PubMedGoogle Scholar
  50. Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. Arthritis Rheum 1990;33:1074–87PubMedCrossRefGoogle Scholar
  51. Liozon E, Roblot P, Paire D, et al Anticardiolipin antibody levels predict flares and relapses in patients with giant-cell (temporal) arteri-tis. A longitudinal study of 58 biopsy-proven cases. Rheumatology (Oxford) 2000;39(10):1089–94CrossRefGoogle Scholar
  52. Lundberg I, Hedfors E. Restricted dose and duration of corticosteroid treatment in patients with polymyalgia rheumatica and temporal arteritis. J Rheumatol 1990;17(10):1340–5PubMedGoogle Scholar
  53. Mahr AD, Jover JA, Spiera RF, et al Adjunctive methotrexate to treat giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum 2007;56(8):2789–97PubMedCrossRefGoogle Scholar
  54. Manna R, Latteri M, Cristiano G, et al Anticardiolipin antibodies in giant cell arteritis and polymyalgia rheumatica: a study of 40 cases. Br J Rheumatol 1998;37(2):208–10PubMedCrossRefGoogle Scholar
  55. Meller J, Strutz F, Siefker U, et al Early diagnosis and follow-up of aortitis with [(18)F]FDG PET and MRI. Eur J Nucl Med Mol Imaging 2003;30(5):730–6PubMedCrossRefGoogle Scholar
  56. Meyer O, Nicaise P, Moreau S, et al Antibodies to cardiolipin and beta 2 glycoprotein I in patients with polymyalgia rheumatica and giant cell arteritis. Rev Rhum Engl Ed 1996;63(4):241–7PubMedGoogle Scholar
  57. Morgan G, Harris E. Non-giant cell temporal arteritis. Arthritis Rheum 1978;21:362–6PubMedCrossRefGoogle Scholar
  58. Nesher G, Berkun Y, Mates M. Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum 2004;50:1332–7PubMedCrossRefGoogle Scholar
  59. Nuenninghoff DM, Hunder GG, Christianson TJ, et al Mortality of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis: a population-based study over 50 years. Arthritis Rheum 2003;48(12):3532–7PubMedCrossRefGoogle Scholar
  60. Olopade, CO, Sekosan, M, Schraufnagel, DE. Giant cell arteritis manifesting as chronic cough and fever of unknown origin. Mayo Clin Proc 1997;72:1048PubMedCrossRefGoogle Scholar
  61. Papadimitraki ED, Kyrmizakis DE, Kritikos I, Boumpas DT. Ear-nose-throat manifestations of autoimmune rheumatic diseases. Clin Exp Rheumatol 2004;22(4):485–94PubMedGoogle Scholar
  62. Paraskevas KI, Boumpas DT, Vrentzos GE, Mikhailidis DP. Oral and ocular/orbital manifestations of temporal arteritis: a disease with deceptive clinical symptoms and devastating consequences. Clin Rheumatol 2007;26(7):1044–8PubMedCrossRefGoogle Scholar
  63. Pipitone N, Boiardi L, Bajocchi G, Salvarani C. Long-term outcome of giant cell arteritis. Clin Exp Rheumatol 2006;24(2 Suppl 41): S65–70PubMedGoogle Scholar
  64. Pipitone N, Boiardi L, Salvarani C. Are steroids alone sufficient for the treatment of giant cell arteritis? Best Pract Res Clin Rheumatol 2005;19(2):277–92PubMedCrossRefGoogle Scholar
  65. Pipitone N, Versari A, Salvarani C. Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford) 2008;47(4):403–8CrossRefGoogle Scholar
  66. Pless M, Rizzo JF III, Lamkin JC, Lessell S. Concordance of bilateral temporal artery biopsy in giant cell arteritis. J Neuroophthalmol 2000;20:216–8PubMedCrossRefGoogle Scholar
  67. Porter MJ. Temporal arteritis presenting as a submandibular swelling. J Laryngol Otol 1990;104(10):819–20PubMedCrossRefGoogle Scholar
  68. Razavi M, Jones, RD, Manzel, et al Steroid-responsive Charles Bonnet syndrome in temporal arteritis. J Neuropsychiatry Clin Neurosci 2004;16:505PubMedCrossRefGoogle Scholar
  69. Regan MJ, Green WR, Stone JH. Ethnic disparity in the incidence of temporal arteritis: a 32-year expereince at an urban medical center. Arthritis Rheum 2000;43:S128Google Scholar
  70. Rojo-Leyva F, Ratliff N, Cosgrove D, et al Study of 52 patients with idiopathic aortitis from a cohort of 1204 surgical cases. Arthritis Rheum 2000;43:901–7PubMedCrossRefGoogle Scholar
  71. Rucker JC, Biousse V, Newman NJ. Ischemic optic neuropathies. Curr Opin Neurol 2004;17(1):27–35PubMedCrossRefGoogle Scholar
  72. Ruiz-Masera JJ, Alamillos-Granados FJ, Dean-Ferrer A, et al Sub-mandibular swelling as the first manifestation of giant cell arteritis. Report of a case. J Craniomaxillofac Surg 1995;23(2):119–21PubMedCrossRefGoogle Scholar
  73. Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 2002a;347(4):261–71CrossRefGoogle Scholar
  74. Salvarani C, Giannini C, Miller DV, et al Giant cell arteritis: involvement of intracranial arteries. Arthritis Rheum 2006;55:985–9PubMedCrossRefGoogle Scholar
  75. Salvarani C, Hunder GG. Giant cell arteritis with a low erythrocyte sedimentation rate: Frequency of occurence in a population-based study. Arthritis Rheum 2001;45:140–5PubMedCrossRefGoogle Scholar
  76. Salvarani C, Silingardi M, Ghirarduzzi A, et al Is duplex ultrasonogra-phy useful for the diagnosis of giant-cell arteritis? Ann Intern Med 2002b;137:232CrossRefGoogle Scholar
  77. Schmidt WA, Kraft HE, Vorpahl K, Volker L, Gromnica-Ihle EJ. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med 1997;337(19):1336–42PubMedCrossRefGoogle Scholar
  78. Seo P, Stone JH. Large-vessel vasculitis. Arthritis Care Res 2002;51: 128–39CrossRefGoogle Scholar
  79. Small P, Brisson M-L. Wegener's granulomatosis presenting as temporal arteritis. Arthritis Rheum 1991;34:220–3PubMedCrossRefGoogle Scholar
  80. Smetana GW, Shmerling RH. Does this patient have temporal arteritis? JAMA 2002;287:92–101PubMedCrossRefGoogle Scholar
  81. Spiera RF, Mitnick HJ, Kupersmith M, et al A prospective, doubleblind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis (GCA). Clin Exp Rheumatol 2001; 19(5):495–501PubMedGoogle Scholar
  82. Stone JH. Hearing voices. Pharos 2006;69:31–2PubMedGoogle Scholar
  83. Stone JH. Antiplatelet versus anticoagulant therapy in patients with giant cell arteritis: which is best? Nat Clin Pract Rheumatol 2007;3: 136–7PubMedCrossRefGoogle Scholar
  84. Stone JH, Francis HW. Immune-mediated inner ear disease. Curr Opin Rheumatol 2000;12(1):32–40PubMedCrossRefGoogle Scholar
  85. Tso E, Flamm SD, White RD, et al Takayasu arteritis: utility and limitations of magnetic resonance imaging in diagnosis and treatment. Arthritis Rheum 2002;46(6):1634–42PubMedCrossRefGoogle Scholar
  86. Wilkinson IM, Russell RW. Arteries of the head and neck in giant cell arteritis: a pathological study to show the pattern of arterial involvement. Arch Neurol 1972;27:378PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2009

Authors and Affiliations

  • James R. Stone
    • 1
  • Misha Pless
    • 1
  • Carlo Salvarani
    • 2
  • Nicolo Pipitone
    • 2
  • Simmons Lessell
    • 3
  • John H. Stone
    • 4
  1. 1.Massachusetts General Hospital Department of PathologyBoston
  2. 2.Arcispedale S Maria Nuova Viale RisorgimentoReggioItaly
  3. 3.Massachusetts Eye & Ear InfirmaryBostonUSA
  4. 4.Massachusetts General HospitalBoston

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