Skip to main content

Advertisement

Log in

Systematic review of the literature and a case report informing biopsy-proven giant cell arteritis (GCA) with normal C-reactive protein

  • Case Based Review
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Giant cell arteritis (GCA) is a vasculitis of large- vessels. A markedly elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels are characteristics of GCA, although temporal artery biopsy remains the gold standard for the diagnosis. We describe a case of biopsy-proven GCA showing a heavy infiltration of CD68 macrophages and CD3 T cells and with normal ESR and CRP levels at diagnosis.

Key points

(1) GCA may occur with normal ESR in a percentage of about 4 to 15 % (although the American College of Rheumatology classification criteria for giant cell arteritis include an ESR of 50 mm/h or more), while it can occur with normal ESR and normal CRP in a percentage of about 0.8 %. So, the clinical suspicion must be confirmed with a positive biopsy.

(2) GCA patients with ESR >40 mm/h are characterized by higher incidence of headache and jaw claudication compared to patients with normal ESR. In our case, it occurred with normal ESR.

(3) Color duplex ultrasonography is a noninvasive, easy, and inexpensive method for supporting a diagnosis of TA, with a high sensitivity and specificity. It can predict which patient will need TAB.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Smetana GW, Shmerling RH (2002) Does this patient have temporal arteritis? JAMA 287(1):92–101

    Article  PubMed  Google Scholar 

  2. Salvarani C, Hunder GG (2001) Giant cell arteritis with low erythrocyte sedimentation rate: frequency of occurrence in a population-based study. Arthritis Care Res 45:140

    Article  CAS  Google Scholar 

  3. Hunder GG (2006) The early history of giant cell arteritis and polymyalgia rheumatica. First descriptions to 1970. Mayo Clin Proc 81(8):1071–1083

    Article  PubMed  Google Scholar 

  4. Hellmann DB (2004) Giant cell arteritis and polymyalgia rheumatica. In: Imboden JB, Hellmann DB, Stone JH (eds) Current rheumatology diagnosis and treatment. McGraw Hill, New York, pp 235–241

    Google Scholar 

  5. Hunder GG, Bloch DA, Michel BA, Stevens MB et al (1990) The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 33:1122–1128

    Article  PubMed  CAS  Google Scholar 

  6. Bhatti MT, Tabandeh H (2001) Giant cell arteritis: diagnosis and management. Curr Opin Ophthalmol 12:393–399

    Article  PubMed  CAS  Google Scholar 

  7. Ezeonyeji Amara N, Borg Frances A, Dasgupta Bhaskar (2011) Delays in recognition and management of giant cell arteritis:results from a retrospective audit. Clin Rheumatol 30:259–262

    Article  PubMed  Google Scholar 

  8. Weyand CM, Goronzy JJ (2003) Giant cell arteritis and polymalgia rheumatic. Ann Intern Med 139:505–515

    PubMed  Google Scholar 

  9. Hall JK, Volpe NJ, Galetta SL, Liu GT et al (2003) The role of unilateral temporal artery biopsy. Ophthalmology 110(3):543–548

    Article  PubMed  Google Scholar 

  10. Nesher G, Shemesh D, Mates M, Sonnenblick M, Abrmowitz HBJ (2002) The predictive value of the halo sign in color Doppler ultrasonography of the temporal arteries for diagnosing giant cell arteritis. Rheumatol 29(6):1224–1226

    Google Scholar 

  11. Karahaliou M, Vaiopoulos G, Papaspyrou S, Kankis M, Revenas K, Sfikakis PP (2006) Colour duplex sonography of temporal arteries before decision for biopsy: a prospective study in 55 patients with suspected giant cell arteritis. Arthritis Res Ther 8(4):R116

    Article  PubMed  Google Scholar 

  12. Habib Hisham M, Essa Ashraf A, Hassan Ayman A (2012) Color duplex ultrasonography of temporal arteries: role in diagnosis and follow-up of suspected cases of temporal arteritis. Clin Rheumatol 31:231–237

    Article  PubMed  Google Scholar 

  13. Wong RL, Korn JH (1986) Temporal arteritis without an elevated erythrocyte sedimentation rate. Case report and review of the literature. Am J Med 80(5):959–964

    Article  PubMed  CAS  Google Scholar 

  14. Myklebust G, Gran JT (1996) A prospective study of 287 patients with polymyalgia rheumatica and temporal arterttis: clinical and laboratory manifestations at onset of disease and at the time of diagnosis. Br J Rheumatol 35:1161–1168

    Article  PubMed  CAS  Google Scholar 

  15. Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback LR (1978) Temporal arteritis: a 25-year epidemiologic, clinical, and pathologic study. Ann Intern Med 88(2):162–167

    PubMed  CAS  Google Scholar 

  16. Parikh M, Miller NR, Lee AG, Savino PJ, Vacarezza MN, Cornblath W, Eggenberger E, Antonio-Santos A, Golnik K, Kardon R, Wall M (2006) Prevalence of a normal C-reactive protein with an elevated erythrocyte sedimentation rate in biopsy-proven giant cell arteritis. Ophthalmology 113(10):1842–1845

    Article  PubMed  Google Scholar 

  17. Kermani TA, Schmidt J, Crowson CS, Ytterberg SR, Hunder GG, Matteson EL, Warrington KJ (2011) Utility of erythrocyte sedimentation rate and C-reactive protein for the diagnosis of giant cell arteritis. Semin Arthritis Rheum 41(6):866–871

    Article  PubMed  Google Scholar 

  18. Weyand CM, Goronzy JJ (1999) Arterial wall injury in giant cell arteritis. Arthritis Rheum 42(5):844–853

    Article  PubMed  CAS  Google Scholar 

  19. Lopez-Diaz MJ, Llorca J, Gonzalez-Juanatey C, Peña-Sagredo JL, Martin J, Gonzalez-Gay MA (2008) The erythrocyte sedimentation rate is associated with the development of visual complications in biopsy-proven giant cell arteritis. Semin Arthritis Rheum 38:116–123

    Article  PubMed  Google Scholar 

  20. Cantini F, Salvarani C, Olivieri I (1998) Erythrocyte sedimentation rate and C-reactive protein in the diagnosis of polymyalgia rheumatica. Ann Intern Med 128(10):873–874

    PubMed  CAS  Google Scholar 

  21. Pepys MB, Hirschfield GM (2003) C-reactive protein: a critical update. J Clin Invest 111(12):1805–1812

    PubMed  CAS  Google Scholar 

  22. Szalai AJ, McCrory MA, Cooper GS, Wu J, Kimberly RP (2002) Association between baseline levels of C-reactive protein (CRP) and a dinucleotide repeat polymorphism in the intron of the CRP gene. Genes Immun 3:14–19

    Article  PubMed  CAS  Google Scholar 

  23. Russell AI, Cunninghame GDS, Shepherd C, Roberton CA, Whittaker J, Meeks J, Powell RJ, Isenberg DA, Walport MJ, Vyse TJ (2004) Polymorphism at the C-reactive protein locus influences gene expression and predisposes to systemic lupus erythematosus. Hum Mol Genet 13(1):137–147

    Article  PubMed  CAS  Google Scholar 

  24. Ellis ME, Ralston S (1983) The ESR in the diagnosis and management of the polymyalgia rheumatica/giant cell arteritis syndrome. Ann Rheum Dis 42:168–170

    Google Scholar 

  25. Branum G, Massey EW, Rice J (1987) Erythrocyte sedimentation rate in temporal arteritis. South Med J 80:1527–1528

    Google Scholar 

  26. Kyle V, Cawston TE, Hazleman BL (1989) Erythrocyte sedimentation rate and C reactive protein in the assessment of polymyalgia rheumatica/giant cell arteritis on presentation and during follow up. Ann Rheum Dis 48:667–671

    Google Scholar 

  27. Jundt JW, Mock D (1991) Temporal arteritis with normal erythrocyte sedimentation rates presenting as occipital neuralgia. Arthritis Rheum 34(2):217–219

    Google Scholar 

  28. Wise CM, Agudelo CA, Chmelewski WL, McKnight KM (1991) Temporal arteritis with low erythrocyte sedimentation rate: a review of five cases. Arthritis Rheum 34:1571–1574

    Google Scholar 

  29. Myklebust G, Gran JT (1996) A prospective study of 287 patients with polymyalgia rheumatica and temporal arteritis: clinical and laboratory manifestations at onset of disease and at the time of diagnosis. Br J Rheumatol 35:1161–1168

    Google Scholar 

  30. Olsson AT, Elling H, Elling P (1997) Frequency of a normal erythrocyte sedimentation rate in patients with active,untreated arteritis temporalis and polymyalgia rheumatica: comment on the articleby Helfgott and Kieval. Arthritis Rheum 40:191–3

    Google Scholar 

  31. von Blotzheim SG, Borruat FX (1996) [Neuro-ophthalmological complications of Horton's disease]. Rev Med Suisse Romande 116:21–25

    Google Scholar 

  32. Martínez-Taboada VM, Blanco R, Armona J, Uriarte E, Figueroa M, Gonzalez-Gay MA, Rodriguez-Valverde V (2000) Giant cell arteritis with an erythrocyte sedimentation rate lower than 50. Clin Rheumatol 19:73–75

    Google Scholar 

  33. Pamuk ON, Dönmez S, Karahan B, Pamuk GE, Cakir N (2009) Giant cell arteritis and polymyalgia rheumatica in northwestern Turkey: Clinical features and epidemiological data. Clin Exp Rheumatol 27:830–833

    Google Scholar 

Download references

Disclosures

None.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to G. F. Ferraccioli.

Rights and permissions

Reprints and permissions

About this article

Cite this article

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 31, 1389–1393 (2012). https://doi.org/10.1007/s10067-012-2031-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-012-2031-3

Keywords

Navigation