eMedicine Specialties > Rheumatology > Vasculitis
Giant Cell Arteritis: Differential Diagnoses & Workup
Updated: Nov 14, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Differential Diagnoses
| Atherosclerosis | Rheumatoid Arthritis |
| Atherosclerotic Disease of the Carotid
Artery | Takayasu Arteritis |
| Cluster headache | Trigeminal neuralgia |
| Fever of Unknown Origin | Wegener Granulomatosis |
| Granulomatous angiitis of the CNS | |
| Migraine headache | |
| Polymyalgia rheumatica |
Other Problems to Be Considered
Sinusitis
Dental problems
Workup
Laboratory Studies
- Blood studies
- The laboratory hallmark of polymyalgia rheumatica and giant cell arteritis (GCA) is an elevation in the acute-phase reactants, ie, ESR and C-reactive protein (CRP). The ESR usually exceeds 50 mm/h and may exceed 100 mm/h. However, an ESR in the low 30s or 20s does not exclude polymyalgia rheumatica or giant cell arteritis if other characteristic clinical or, in the case of giant cell arteritis, pathological, features are present.14
- Normocytic normochromic anemia and thrombocytosis occur in approximately 50% of patients with polymyalgia rheumatica and are good guides to the state of inflammation. These findings are also common in giant cell arteritis.
- In both polymyalgia rheumatica and giant cell arteritis, the frequency of rheumatoid factor, antinuclear antibodies, and other autoreactive antibodies is not higher than that of age-matched controls. Complement levels are normal, and cryoglobulins and monoclonal immunoglobulins are absent.
- Findings from liver function tests, especially the alkaline phosphatase level, may be elevated in approximately one third of patients, most commonly in giant cell arteritis rather than pure polymyalgia rheumatica.
- Muscle enzyme levels (eg, creatine kinase, aldolase) are normal in both polymyalgia rheumatica and giant cell arteritis.
Imaging Studies
- Researchers have recently explored the role of color duplex ultrasonography in the diagnosis of giant cell arteritis. A hypoechoic halo around the temporal artery lumen on color duplex sonograms was reported in 22 of 30 patients (73%) with biopsy-proven giant cell arteritis.15 The halo was observed bilaterally in 17 of these patients and disappeared at a mean of 16 days after the initiation of corticosteroid therapy. The presence of the halo had a sensitivity of 73% and was 100% specific for giant cell arteritis. The halo represents edema in the artery wall. Although some centers consider this finding a major decision-maker in their diagnostic equation, others do not believe that such a halo is definitive for giant cell arteritis. Superficial temporal artery biopsy remains the standard for diagnosis of giant cell arteritis (see Procedures).
- Thoracic or abdominal ultrasonography may be helpful for diagnosing and monitoring patients with aortic aneurysms.
- In giant cell arteritis, temporal artery arteriography has no diagnostic value and does not aid in predicting the proper biopsy site for the temporal artery.
- Polymyalgia rheumatica has no characteristic imaging abnormalities. However, recent MRI findings of tendinitis and bursitis in the shoulders of patients with polymyalgia rheumatica support the soft tissues as significant sites of inflammation and sources of symptoms in polymyalgia rheumatica.
Other Tests
- Electromyographic studies
- Results are within normal limits.
- This study is rarely needed in patients with a clinical presentation of polymyalgia rheumatica.
- Synovial fluid and tissue studies
- Leukocyte counts in joint fluid, which are rarely available for study, range from 1000-8000/µL, with a preponderance of mononuclear cells.
- Synovial biopsy findings, when available, reveal mild synovial proliferation with slight lymphocytic infiltration.16
Procedures
- Muscle biopsy
- Histology findings are not diagnostic in polymyalgia rheumatica; type II muscle fiber atrophy probably represents disuse.
- This study is not indicated because patients with polymyalgia rheumatica do not present with weakness or muscle enzyme abnormalities.
- Temporal artery biopsy
- Consider this biopsy if a patient with polymyalgia rheumatica has symptoms or signs suggestive of giant cell arteritis or is unresponsive to 15 mg prednisone daily. It may also be indicated in the workup of an elderly patient with fever of unknown origin with a high ESR in whom infection and malignancy testing has been unrevealing.
- Temporal artery biopsy can be performed on an ambulatory basis and, if findings are positive, can dramatically diminish the diagnostic studies otherwise needed in evaluating systemically ill patients with an elevated ESR.
- Several clinical studies demonstrate that the likelihood of positive findings after temporal artery biopsy in patients with giant cell arteritis polymyalgia rheumatica is greatly enhanced if temporal artery pulses are absent or diminished, even in the absence of other localizing signs. The presence of a nonspecific headache may also increase the yield.
- No difference is evident in the degree of ESR elevation, the presence of minor visual symptoms, sex, age, or the duration of symptoms among patients with polymyalgia rheumatica who do not have giant cell arteritis; furthermore, approximately 10% of patients with polymyalgia rheumatica and localized temporal artery signs have negative biopsy findings.
- Strategies for planning temporal artery biopsies in patients suggested to have giant cell arteritis include the following:
- Always perform the biopsy on the temporal artery on the symptomatic side of the head.
- If a specific part of the artery is tender, beaded, or inflamed, include that area in the biopsy. No information exists on whether the artery trunk or a distal branch specimen is best. Take at least 2-3 cm of the artery. Make multiple sections because the process may be segmental.
- Studies performed during the 1970s and 1980s show that bilateral temporal artery biopsies can increase the sensitivity of diagnosing giant cell arteritis by 11-60%. However, recent studies have found that bilateral biopsies do not increase the diagnostic yield in the vast majority of patients (99%).17,18 However, questions have been raised about the use of steroid treatment for giant cell arteritis in the absence of positive biopsy results.19
- Results of temporal artery biopsies remain positive for characteristic giant cell arteritis pathology after as long as 4 weeks while high doses of corticosteroids are administered. Thus, the physician should never delay the institution of steroids because of fear of ruining the chances of finding an inflamed artery on a biopsy sample.
Histologic Findings
An inflammatory infiltrate, predominantly of mononuclear cells, usually involves the entire vessel wall (ie, panarteritis). Fragmentation of the internal elastic lamina is characteristic. Fibrinoid necrosis is not a feature of the lesion. Giant cells are commonly present, and they often seem to engulf parts of the internal elastic lamina. The giant cells are difficult to find in some cases, and their absence does not exclude the diagnosis. Intimal proliferation is often marked, is a nonspecific feature in this age group, and does not suggest past or present arteritis if found alone. When giant cell arteritis involves larger vessels, the lesions are indistinguishable from those observed in Takayasu arteritis.
More on Giant Cell Arteritis |
| Overview: Giant Cell Arteritis |
Differential Diagnoses & Workup: Giant Cell Arteritis |
| Treatment & Medication: Giant Cell Arteritis |
| Follow-up: Giant Cell Arteritis |
| References |
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References
Hunder GG. The early history of giant cell arteritis and polymyalgia rheumatica: first descriptions to 1970. Mayo Clin Proc. Aug 2006;81(8):1071-83. [Medline].
Barber HS. Myalgic syndrome with constitutional effects; polymyalgia rheumatica. Ann Rheum Dis. Jun 1957;16(2):230-7. [Medline].
Cid MC, Cebrian M, Font C, et al. Cell adhesion molecules in the development of inflammatory infiltrates in giant cell arteritis: inflammation-induced angiogenesis as the preferential site of leukocyte-endothelial cell interactions. Arthritis Rheum. Jan 2000;43(1):184-94. [Medline].
Goodwin JS. Progress in gerontology: polymyalgia rheumatica and temporal arteritis. J Am Geriatr Soc. May 1992;40(5):515-25. [Medline].
Salvarani C, Gabriel SE, O'Fallon WM, Hunder GG. The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern. Ann Intern Med. Aug 1 1995;123(3):192-4. [Medline].
Smeeth L, Cook C, Hall AJ. Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990-2001. Ann Rheum Dis. Aug 2006;65(8):1093-8. [Medline].
Bosley TM, Riley FC. Giant cell arteritis in Saudi Arabia. Int Ophthalmol. 1998;22(1):59-60. [Medline].
González-Gay MA, García-Porrúa C, Llorca J, Hajeer AH, Brañas F, Dababneh A, et al. Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. Medicine (Baltimore). Sep 2000;79(5):283-92. [Medline].
Liu NH, LaBree LD, Feldon SE, Rao NA. The epidemiology of giant cell arteritis : a 12-year retrospective study. Ophthalmology. Jun 2001;108(6):1145-9. [Medline].
Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. Aug 1990;33(8):1122-8. [Medline].
Cid MC, García-Martínez A, Lozano E, Espígol-Frigolé G, Hernández-Rodríguez J. Five clinical conundrums in the management of giant cell arteritis. Rheum Dis Clin North Am. Nov 2007;33(4):819-34, vii. [Medline].
Liang GC, Simkin PA, Hunder GG, et al. Familial aggregation of polymyalgia rheumatica and giant cell arteritis. Arthritis Rheum. Jan-Feb 1974;17(1):19-24. [Medline].
Russo MG, Waxman J, Abdoh AA, Serebro LH. Correlation between infection and the onset of the giant cell (temporal) arteritis syndrome. A trigger mechanism?. Arthritis Rheum. Mar 1995;38(3):374-80. [Medline].
Wise CM, Agudelo CA, Chmelewski WL, McKnight KM. Temporal arteritis with low erythrocyte sedimentation rate: a review of five cases. Arthritis Rheum. Dec 1991;34(12):1571-4. [Medline].
Schmidt WA, Kraft HE, Vorpahl K, et al. Color duplex ultrasonography in the diagnosis of temporal arteritis. N Engl J Med. Nov 6 1997;337(19):1336-42. [Medline].
Meliconi R, Pulsatelli L, Uguccioni M, et al. Leukocyte infiltration in synovial tissue from the shoulder of patients with polymyalgia rheumatica. Quantitative analysis and influence of corticosteroid treatment. Arthritis Rheum. Jul 1996;39(7):1199-207. [Medline].
Boyev LR, Miller NR, Green WR. Efficacy of unilateral versus bilateral temporal artery biopsies for the diagnosis of giant cell arteritis. Am J Ophthalmol. Aug 1999;128(2):211-5. [Medline].
Hall JK, Volpe NJ, Galetta SL, Liu GT, Syed NA, Balcer LJ. The role of unilateral temporal artery biopsy. Ophthalmology. Mar 2003;110(3):543-8; discussion 548. [Medline].
Lenton J, Donnelly R, Nash JR. Does temporal artery biopsy influence the management of temporal arteritis?. QJM. Jan 2006;99(1):33-6. [Medline].
Mahr AD, Jover JA, Spiera RF, Hernández-García C, Fernández-Gutiérrez B, Lavalley MP, et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum. Aug 2007;56(8):2789-97. [Medline].
De Silva M, Hazleman BL. Azathioprine in giant cell arteritis/polymyalgia rheumatica: a double-blind study. Ann Rheum Dis. Feb 1986;45(2):136-8. [Medline].
Hoffman GS, Cid MC, Rendt-Zagar KE, Merkel PA, Weyand CM, Stone JH, et al. Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial. Ann Intern Med. May 1 2007;146(9):621-30. [Medline].
Nesher G, Berkun Y, Mates M, Baras M, Rubinow A, Sonnenblick M. Low-dose aspirin in the treatment of giant cell arteritis. Arthritis and Rheumatism. Apr,2004;50:1026-1027. [Medline].
Narvaez J, Nolla-Sole JM, Clavaguera MT, et al. Longterm therapy in polymyalgia rheumatica: effect of coexistent temporal arteritis. J Rheumatol. Sep 1999;26(9):1945-52. [Medline].
Alpers CE, Davis CL, Barr D, et al. Identification of platelet-derived growth factor A and B chains in human renal vascular rejection. Am J Pathol. Feb 1996;148(2):439-51. [Medline].
Bignon JD, Ferec C, Barrier J, et al. HLA class II genes polymorphism in DR4 giant cell arteritis patients. Tissue Antigens. Nov 1988;32(5):254-8. [Medline].
Bjornsson J. Clues to the pathogenesis of giant cell arteritis from the study of the vessel wall. Arthritis Care Res. Oct 2000;13(5):249-51. [Medline].
Blockmans D, Ceuninck L, Vanderschueren S, et al. Repetitive (18)F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: A prospective study of 35 patients. Arthritis Rheum. Feb 2006;55(1):131-7. [Medline].
Bongartz T, Matteson EL. Large-vessel involvement in giant cell arteritis. Curr Opin Rheumatol. Jan 2006;18(1):10-7. [Medline].
Both M, Aries PM, Muller-Hulsbeck S, et al. Balloon angioplasty of upper extremity arteries in patients with extracranial giant cell arteritis. Ann Rheum Dis. Feb 7 2006;[Medline].
Brack A, Martinez-Taboada V, Stanson A, et al. Disease pattern in cranial and large-vessel giant cell arteritis. Arthritis Rheum. Feb 1999;42(2):311-7. [Medline].
Cantini F, Salvarani C, Olivieri I, et al. Inflamed shoulder structures in polymyalgia rheumatica with normal erythrocyte sedimentation rate. Arthritis Rheum. May 2001;44(5):1155-9. [Medline].
Chan JW. Acute monocular visual loss in carcinomatous hypertrophic pachymeningitis mimicking giant cell arteritis. Rheumatol Int. Dec 9 2005;1-2. [Medline].
Dababneh A, Gonzalez-Gay MA, Garcia-Porrua C, et al. Giant cell arteritis and polymyalgia rheumatica can be differentiated by distinct patterns of HLA class II association. J Rheumatol. Nov 1998;25(11):2140-5. [Medline].
de Castro LE, Petersen AM, Givre SJ, et al. Herpes zoster ophthalmicus: presenting as giant-cell arteritis. Clin Experiment Ophthalmol. Dec 2005;33(6):636-8. [Medline].
Fauchald P, Rygvold O, Oystese B. Temporal arteritis and polymyalgia rheumatica. Clinical and biopsy findings. Ann Intern Med. Dec 1972;77(6):845-52. [Medline].
Fox GN. Giant cell arteritis. CMAJ. Dec 6 2005;173(12):1490. [Medline].
Ghanchi FD, Dutton GN. Current concepts in giant cell (temporal) arteritis. Surv Ophthalmol. Sep-Oct 1997;42(2):99-123. [Medline].
Glazunov AV, Zhiliaev EV, Toldieva FA, Dzhanashiia PKh. [Polymyalgia rheumatica and giant-cell arteritis]. Klin Med (Mosk). 2005;83(11):39-45. [Medline].
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). Sep 2005;84(5):277-90. [Medline].
Gonzalez-Juanatey C, Llorca J, Garcia-Porrua C, et al. Steroid therapy improves endothelial function in patients with biopsy-proven giant cell arteritis. J Rheumatol. Jan 2006;33(1):74-8. [Medline].
Hachulla E, Boivin V, Pasturel-Michon U, et al. Prognostic factors and long-term evolution in a cohort of 133 patients with giant cell arteritis. Clin Exp Rheumatol. Mar-Apr 2001;19(2):171-6. [Medline].
Hall S, Hunder GG. Is temporal artery biopsy prudent?. Mayo Clin Proc. Nov 1984;59(11):793-6. [Medline].
Hamilton CR Jr, Shelley WM, Tumulty PA. Giant cell arteritis: including temporal arteritis and polymyalgia rheumatica. Medicine (Baltimore). Jan 1971;50(1):1-27. [Medline].
Haworth S, Ridgeway J, Stewart I, et al. Polymyalgia rheumatica is associated with both HLA-DRB1*0401 and DRB1*0404. Br J Rheumatol. Jul 1996;35(7):632-5. [Medline].
Hernandez-Rodriguez J, Garcia-Martinez A, Espigol-Frigole G, et al. Sustained spontaneous clinical remission in giant cell arteritis: Report of two cases with long-term followup. Arthritis Rheum. Feb 15 2006;55(1):160-162. [Medline].
Herve F, Choussy V, Janvresse A, et al. [Aortic involvement in giant cell arteritis. A prospective follow-up of 11 patients using computed tomography.]. Rev Med Interne. Dec 9 2005;[Medline].
Hollenhorst RW, Brown JR, Wagener HP, Shick RM. Neurologic aspects of temporal arteritis. Neurology. May 1960;10:490-8. [Medline].
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. Jan 16 2001;134(2):106-14. [Medline].
Kaiser M, Weyand CM, Bjornsson J, Goronzy JJ. Platelet-derived growth factor, intimal hyperplasia, and ischemic complications in giant cell arteritis. Arthritis Rheum. Apr 1998;41(4):623-33. [Medline].
Klein RG, Campbell RJ, Hunder GG, Carney JA. Skip lesions in temporal arteritis. Mayo Clin Proc. AUG 1976;51(8):504-10. [Medline].
Limas-Banos I, Seijo-Martinez M. [Giant cell arteritis in association with acute multifocal stroke.]. Rev Neurol. Feb 1-15 2006;42(3):189-91. [Medline].
Martin J, Perez-Armengol C, Miranda-Filloy JA, et al. Lack of association of a functional -94ins/delATTG NFKB1 promoter polymorphism with susceptibility and clinical expression of biopsy-proven giant cell arteritis in northwest Spain. J Rheumatol. Feb 2006;33(2):285-8. [Medline].
Mecklenburg I, Brumberger V, Burchardi C, et al. Hepatic involvement in a patient with giant cell arteritis. Dig Dis Sci. Jan 2006;51(1):39-40. [Medline].
Myklebust G, Wilsgaard T, Jacobsen BK, Gran JT. Causes of death in polymyalgia rheumatica. A prospective longitudinal study of 315 cases and matched population controls. Scand J Rheumatol. 2003;32(1):38-41. [Medline].
O'Duffy JD, Wahner HW, Hunder GG. Joint imaging in polymyalgia rheumatica. Mayo Clin Proc. Aug 1976;51(8):519-24. [Medline].
Patel AD, Varma R. Giant cell arteritis. CMAJ. Dec 6 2005;173(12):1490.
Pfadenhauer K, Rull T. Ultrasonographic and FDG-PET imaging in active giant cell arteritis of the carotid arteries. Vasa. Nov 2005;34(4):269-71. [Medline].
Ripoll JM, Zaragoza JM, Banuls SR, Sanchis AM. First-time manifestation of giant-cell arteritis during methotrexate treatment. J Clin Rheumatol. Aug 2005;11(4):240. [Medline].
Roth AM, Milsow L, Keltner JL. The ultimate diagnoses of patients undergoing temporal artery biopsies. Arch Ophthalmol. Jun 1984;102(6):901-3. [Medline].
Sailler LJ, Porte L, Ollier SM, et al. Giant cell arteritis and spinal cord compression: an overlap syndrome?. Mayo Clin Proc. Jan 2006;81(1):89-91. [Medline].
Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med. Jul 25 2002;347(4):261-71. [Medline].
Salvarani C, Cantini F, Olivieri I, et al. Proximal bursitis in active polymyalgia rheumatica. Ann Intern Med. Jul 1 1997;127(1):27-31. [Medline].
Salvarani C, Hunder GG. Giant cell arteritis with low erythrocyte sedimentation rate: frequency of occurence in a population-based study. Arthritis Rheum. Apr 2001;45(2):140-5. [Medline].
Snow MH, Radio S, Mikuls TR. Disseminated giant cell arteritis with inflammatory arthritis and C-ANCA. J Clin Rheumatol. Aug 2005;11(4):216-8. [Medline].
Sorensen S, Lorenzen I. Giant-cell arteritis, temporal arteritis and polymyalgia rheumatica. A retrospective study of 63 patients. Acta Med Scand. 1977;201(3):207-13. [Medline].
Varnholt H. Giant cell arteritis. CMAJ. Dec 6 2005;173(12):1490. [Medline].
Weyand CM, Goronzy JJ. Arterial wall injury in giant cell arteritis. Arthritis Rheum. May 1999;42(5):844-53. [Medline].
Weyand CM, Hicok KC, Hunder GG, Goronzy JJ. Tissue cytokine patterns in patients with polymyalgia rheumatica and giant cell arteritis. Ann Intern Med. Oct 1 1994;121(7):484-91. [Medline].
Weyand CM, Ma-Krupa W, Pryshchep O, et al. Vascular dendritic cells in giant cell arteritis. Ann N Y Acad Sci. Dec 2005;1062:195-208. [Medline].
Further Reading
Keywords
giant cell arteritis, temporal arteritis, GCA, cranial arteritis, polymyalgia rheumatica, PMR, occult giant cell arteritis, aching syndrome, Horton disease, Horton's disease, intimal hyperplasia, vasculopathy, ischemic optic retinopathy, stenotic lesions, ischemic optic retinopathy, vision loss, visual defects, fever of unknown origin, systemic vasculitis, granulomatous arteritis, limb claudication
Differential Diagnoses & Workup: Giant Cell Arteritis