Skip to main content
SpringerLink
Log in

Giant Cell Arteritis and Polymyalgia Rheumatica: an Update

  • Vasculitis (L Espinoza, Section Editor)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are two closely related diseases in people aged 50 years and older, which are more frequently observed in Western countries. Despite being common entities, concern still exists about the epidemiology, pathogenesis, and diagnosis of both entities. New imaging techniques, such as 18 fluorodeoxyglucose-positron emission tomography, have proved to be useful in detecting large-vessel involvement in GCA. Corticosteroids are the cornerstone of the therapy in GCA and PMR. Relapses are frequent in these conditions. Unlike methotrexate and tumor necrosis factor-α antagonists, anti-interleukin-6 receptor therapy appears to be useful in patients with GCA and PMR who are refractory to corticosteroids. This review summarizes recent studies on GCA and PMR.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, Miranda-Filloy JA, Gonzalez-Juanatey C, Martin J, et al. Epidemiology of giant cell arteritis and polymyalgia rheumatica. Arthritis Rheum. 2009;61:1454–612.

    Article  PubMed  Google Scholar 

  2. Gonzalez-Gay MA. Giant cell arteritis and polymyalgia rheumatica: two different but often overlapping conditions. Semin Arthritis Rheum. 2004;33:289–93.

    Article  PubMed  Google Scholar 

  3. Gonzalez-Gay MA, Miranda-Filloy JA, Lopez-Diaz MJ, Perez-Alvarez R, Gonzalez-Juanatey C, Sanchez-Andrade A, et al. Giant cell arteritis in northwestern Spain: a 25-year epidemiologic study. Medicine (Baltimore). 2007;86:61–88.

    Article  Google Scholar 

  4. Petri H, Nevitt A, Sarsour K, Napalkov P, Collinson N. Incidence of giant cell arteritis and characteristics of patients: data-driven analysis of comorbidities. Arthritis Care Res (Hoboken). 2014. doi:10.1002/acr.22429. The incidence of GCA is higher in women, in particular in the 70 to 79 years old age group.

    Google Scholar 

  5. Dunstan E, Lester SL, Rischmueller M, Dodd T, Black R, Ahern M, et al. Epidemiology of biopsy-proven giant cell arteritis in South Australia. Intern Med J. 2014;44:32–9. Clinical features is Australian individuals with GCA is similar to that previously reported in Western countries.

    Article  CAS  PubMed  Google Scholar 

  6. Alba MA, Mena-Madrazo JA, Reyes E, Flores-Suárez LF. Giant cell arteritis in Mexican patients. J Clin Rheumatol. 2012;18:1–7. The incidence of GCA in Hispanic is low.

    Article  PubMed  Google Scholar 

  7. González-Gay MA, Amoli MM, Garcia-Porrua C, Ollier WE. Genetic markers of disease susceptibility and severity in giant cell arteritis and polymyalgia rheumatica. Semin Arthritis Rheum. 2003;33:38–48.

    Article  PubMed  Google Scholar 

  8. Serrano A, Márquez A, Mackie SL, Carmona FD, Solans R, Miranda-Filloy JA, et al. Identification of the PTPN22 functional variant R620W as susceptibility genetic factor for giant cell arteritis. Ann Rheum Dis. 2013;72:1882–6. PTPN22 R620W functional variant has been implicated in the susceptibility to several autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus. The present study also confirms its implication in GCA. These findings indicate that PTPN22 may represent a common pathway for different autoimmune diseases.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  9. Carmona FD, González-Gay MA, Martín J. Genetic component of giant cell arteritis. Rheumatology (Oxford). 2014;53:6–18. Besides the well-known association with genes located in the HLA region, other gene polymorphisms located outside this region also account for the susceptibility to GCA.

    Article  CAS  Google Scholar 

  10. Weyand CM, Goronzy JJ. Arterial wall injury in giant cell arteritis. Arthritis Rheum. 1999;42:844.

    Article  CAS  PubMed  Google Scholar 

  11. Dejaco C, Duftner C, Al-Massad J, Wagner AD, Park JK, Fessler J, et al. NKG2D stimulated T-cell autoreactivity in giant cell arteritis and polymyalgia rheumatica. Ann Rheum Dis. 2013;72:1852–9. This study highlights the relevance of T cells and the implication of NKG2D in the pathogenesis of GCA and PMR.

    Article  CAS  PubMed  Google Scholar 

  12. Samson M, Audia S, Fraszczak J, Trad M, Ornetti P, Lakomy D, et al. Th1 and Th17 lymphocytes expressing CD161 are implicated in giant cell arteritis and polymyalgia rheumatica pathogenesis. Arthritis Rheum. 2012;64:3788–98. Although the suppressive activity of circulating Treg cells is not altered, the number of circulating Treg cells is decreased in patients with GCA or PMR. The numbers of Th17 lymphocytes are significantly increased in patients with GCA and PMR.

    Article  CAS  PubMed  Google Scholar 

  13. van der Geest KS, Abdulahad WH, Chalan P, Rutgers A, Horst G, Huitema MG, et al. Disturbed B cell homeostasis in newly diagnosed giant cell arteritis and polymyalgia rheumatica. Arthritis Rheumatol. 2014;66:1927–38. Patients newly diagnosed as having GCA or PMR exhibit decreased numbers of circulating B cells compared to healthy controls. B cell numbers recover following therapy in patients with GCA and PMR.

    Article  PubMed  Google Scholar 

  14. Gonzalez-Gay M. The diagnosis and management of patients with giant cell arteritis. J Rheumatol. 2005;32:1186–8.

    PubMed  Google Scholar 

  15. 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;337:1336–42.

    Article  CAS  PubMed  Google Scholar 

  16. Arida A, Kyprianou M, Kanakis M, Sfikakis PP. The diagnostic value of ultrasonography-derived edema of the temporal artery wall in giant cell arteritis: a second meta-analysis. BMC Musculoskelet Disord. 2010;11:44.

    Article  PubMed Central  PubMed  Google Scholar 

  17. Germanò G, Muratore F, Cimino L, Lo Gullo A, Possemato N, Macchioni P, et al. Is colour duplex sonography-guided temporal artery biopsy useful in the diagnosis of giant cell arteritis? A randomized study. Rheumatology (Oxford) 2014 Jun 17. pii: keu241. [Epub ahead of print] Interesting study that indicates that color duplex US of the temporal artery does not improve the sensitive of TAB to make a diagnosis of GCA. The results, rather than reducing the potential usefulness of color duplex US for the diagnosis of GCA, highlight the relevance of an adequate clinical examination to proceed to TAB when GCA is suspected.

  18. Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, Gonzalez-Louzao C, Rodriguez-Ledo P. Biopsy-negative giant cell arteritis: clinical spectrum and predictive factors for positive temporal artery biopsy. Semin Arthritis Rheum. 2001;30:249–56.

    Article  CAS  PubMed  Google Scholar 

  19. Cavazza A, Muratore F, Boiardi L, Restuccia G, Pipitone N, Pazzola G, et al. Inflamed temporal artery: histologic findings in 354 biopsies, with clinical correlations. Am J Surg Pathol. 2014;38:1360–70. Study on 322 temporal artery biopsies. It discloses the wide spectrum of inflammatory lesions found in TAB. It also confirmed that some conditions, in particular systemic vasculitides different from GCA, may cause inflammation of the temporal artery.

    Article  PubMed  Google Scholar 

  20. Sakellariou G, Iagnocco A, Riente L, Ceccarelli F, Carli L, Di Geso L, et al. Ultrasound imaging for the rheumatologist XLIII. Ultrasonographic evaluation of shoulders and hips in patients with polymyalgia rheumatica: a systematic literature review. Clin Exp Rheumatol. 2013;31:1–7. Very elegant review article that indicates that in patients with suggestive clinical features of PMR, a shoulder US, in particular when findings are bilateral, is useful to support a diagnosis of PMR.

  21. Gonzalez-Gay MA, Garcia-Porrua C, Piñeiro A, Pego-Reigosa R, Llorca J, Hunder GG. Aortic aneurysm and dissection in patients with biopsy-proven giant cell arteritis from northwestern Spain: a population-based study. Medicine (Baltimore). 2004;83:335–41.

    Article  Google Scholar 

  22. 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;47:403–8.

    Article  CAS  Google Scholar 

  23. Puppo C, Massollo M, Paparo F, Camellino D, Piccardo A, Shoushtari Zadeh Naseri M, et al. Giant cell arteritis: a systematic review of the qualitative and semiquantitative methods to assess vasculitis with 18F-fluorodeoxyglucose positron emission tomography. Biomed Res Int. 2014;2014:574248. 18 F-FDG- PET is an effective tool for the diagnosis, grading, and follow-up of patients affected by GCA involving the aorta and its proximal branches. In this exhaustive review the authors assessed the qualitative and quantitative methods used for the assessment of vasculitis by 18-FDG-PET. They concluded that the qualitative methods may be more specific than semiquantitative ones, but they have lower sensitivity.

  24. Gonzalez-Gay MA, Martinez-Dubois C, Agudo M, Pompei O, Blanco R, Llorca J. Giant cell arteritis: epidemiology, diagnosis, and management. Curr Rheumatol Rep. 2010;12:436–42.

    Article  PubMed  Google Scholar 

  25. Bejerano C, Blanco R, González-Vela C, Pérez-Martín I, Martinez-Rodriguez I, Jimenez-Bonilla J, et al. Polymyalgia rheumatica as presenting manifestation of vasculitis involving the lower extremities in a patient with ulcerative colitis. Clin Exp Rheumatol. 2012;30(1 Suppl 70):S110–3.

  26. Bejerano C, Blanco R, González-Vela C, Agüero R, Carril JM, González-Gay MA. Refractory polymyalgia rheumatica as presenting manifestation of large-vessel vasculitis associated to sarcoidosis. Successful response to adalimumab. Clin Exp Rheumatol. 2012;30(1 Suppl 70):S94–7.

    PubMed  Google Scholar 

  27. Martínez-Rodríguez I, Martínez-Amador N, Banzo I, Quirce R, Jiménez-Bonilla J, De Arcocha-Torres M, et al. Assessment of aortitis by semiquantitative analysis of 180-min (18)F-FDG PET/CT acquisition images. Eur J Nucl Med Mol Imaging. 2014;41(12):2319–24. Prospective study that included 43 consecutive patients assessed by semiquantitative analysis of PET-CT because of suspected large vessel vasculitis. The authors found that images acquired 180 min after 18 F-FDG injection and a target to background ratio index of 1.34 showed very high accuracy and are strongly recommended for the diagnosis of aortitis in the clinical setting.

  28. Baerlecken NT, Linnemann A, Gross WL, Moosig F, Vazquez-Rodriguez TR, Gonzalez-Gay MA, et al. Association of ferritin autoantibodies with giant cell arteritis/polymyalgia rheumatica. Ann Rheum Dis. 2012;71:943–7. Ferritin autoantibodies may be useful as a diagnostic and activity marker of PMR and GCA.

  29. Große K, Schmidt RE, Witte T, Baerlecken NT. Epitope mapping of antibodies against ferritin heavy chain in giant cell arteritis and polymyalgia rheumatica. Scand J Rheumatol. 2013;42:215–9. Potential diagnostic test for GCA and PMR can be improved by combining three human ferritin peptide antibodies.

    Article  PubMed  Google Scholar 

  30. Weyand CM, Goronzy JJ. Clinical practice. Giant-cell arteritis and polymyalgia rheumatica. N Engl J Med. 2014;371:50–7. Review article that discussed preexisting information on the pathophysiology, diagnosis, clinical features and treatment of GCA. This review also points out some areas of uncertainty in GCA and PMR.

    Article  PubMed Central  PubMed  Google Scholar 

  31. Figus M, Talarico R, Posarelli C, d’Ascanio A, Elefante E, Bombardieri S. Ocular involvement in giant cell arteritis. Clin Exp Rheumatol. 2013;31(1 Suppl 75):S96. Cohort study that confirms the high frequency of visual manifestations in patients with GCA. Early recognition and rapid onset of corticosteroid therapy is of main importance to prevent irreversible visual loss.

    CAS  PubMed  Google Scholar 

  32. Gonzalez-Gay MA, Blanco R, Rodriguez-Valverde V, Martínez-Taboada VM, Delgado-Rodriguez M, Figueroa M, et al. Permanent visual loss and cerebrovascular accidents in giant cell arteritis: predictors and response to treatment. Arthritis Rheum. 1998;41:1497–504.

  33. Muratore F, Pazzola G, Pipitone N, Boiardi L, Salvarani C. Large-vessel involvement in giant cell arteritis and polymyalgia rheumatica. Clin Exp Rheumatol. 2014;32(3 Suppl 82):S106–S11. An extremely interesting and informative review article that highlights important points on the diagnosis and management of patients with large vessel involvement in the setting of GCA.

    PubMed  Google Scholar 

  34. Muratore F, Kermani TA, Crowson CS, Green AB, Salvarani C, Matteson EL, et al. Large-vessel giant cell arteritis: a cohort study. Rheumatology (Oxford). 2014 Sep 5. pii: keu329. [Epub ahead of print] The clinical spectrum of patients with large vessel involvement in the setting of GCA is different from that found in patients presenting with typical cranial manifestations of GCA. Patients with large vessel involvement have less frequency of severe visual complication but they experience more relapses and have higher corticosteroid requirements than those presenting with classic cranial manifestations.

  35. Talarico R, Boiardi L, Pipitone N, d'Ascanio A, Stagnaro C, Ferrari C, et al. Isolated aortitis versus giant cell arteritis: are they really two sides of the same coin? Clin Exp Rheumatol. 2014;32(3 Suppl 82):S55–8. An interesting study that confirms that the clinical spectrum of patients with isolated aortitis is different from that observed in those with cranial manifestations of GCA.

  36. Salvarani C, Barozzi L, Boiardi L, Pipitone N, Bajocchi GL, Macchioni PL, et al. Lumbar interspinous bursitis in active polymyalgia rheumatica. Clin Exp Rheumatol. 2013;31:526–31. Inflammation of lumbar bursae may be responsible for the low back pain reported by patients with PMR.

  37. Martinez-Lado L, Calviño-Díaz C, Piñeiro A, Dierssen T, Vazquez-Rodriguez TR, Miranda-Filloy JA, et al. Relapses and recurrences in giant cell arteritis: a population-based study of patients with biopsy-proven disease from northwestern Spain. Medicine (Baltimore). 2011;90:186–93.

  38. Alba MA, García-Martínez A, Prieto-González S, Tavera-Bahillo I, Corbera-Bellalta M, Planas-Rigol E, et al. Relapses in patients with giant cell arteritis: prevalence, characteristics, and associated clinical findings in a longitudinally followed cohort of 106 patients. Medicine (Baltimore). 2014;93:194–201. Relapses are common in patients with GCA. They occur predominantly within the first 2 years of treatment. PMR symptoms are common with relapses but blindness is exceptional.

  39. Lee JH, Choi ST, Kim JS, Yoon BY, Kwok SK, Kim HS, et al. Clinical characteristics and prognostic factors for relapse in patients with polymyalgia rheumatica (PMR). Rheumatol Int. 2013;33:1475–80. As observed in GCA, relapses are also common in patients with PMR.

  40. Yates M, Loke YK, Watts RA, MacGregor AJ. Prednisolone combined with adjunctive immunosuppression is not superior to prednisolone alone in terms of efficacy and safety in giant cell arteritis: meta-analysis. Clin Rheumatol. 2014;33:227–36. Randomized controlled trials on MTX or anti-TNF-alpha drugs versus corticosteroids do not show that the use of these adjunctive drugs may improve the outcome of GCA.

    Article  CAS  PubMed  Google Scholar 

  41. 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. 2007;56:2789–97.

  42. 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. Small series of patients that showed a beneficial effect of leflunomide in GCA and PMR.

    Article  PubMed Central  PubMed  Google Scholar 

  43. Loricera J, Blanco R, Castañeda S, Humbría A, Ortego-Centeno N, Narváez J, et al. Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol. 2014;32(3 Suppl 82):S79. Tocilizumab may be effective in patients with inflammatory aortitis refractory to corticosteroids or to other biologic immunosuppressive drugs.

  44. Macchioni P, Boiardi L, Catanoso M, Pulsatelli L, Pipitone N, Meliconi R, et al. Tocilizumab for polymyalgia rheumatica: report of two cases and review of the literature. Semin Arthritis Rheum. 2013;43:113–8. Tocilizumab has proved to be effective in some cases of PMR.

  45. Gonzalez-Gay MA, Lopez-Diaz MJ, Martinez-Lado L, Peña-Sagredo JL, Lopez-Agreda H, Miranda-Filloy JA, et al. Cancer in biopsy-proven giant cell arteritis. A population-based study. Semin Arthritis Rheum. 2007;37:156–63.

  46. Kermani TA, Schäfer VS, Crowson CS, Hunder GG, Gabriel SE, Ytterberg SR, et al. Malignancy risk in patients with giant cell arteritis: a population-based cohort study. Arthritis Care Res. 2010;62(2):149–54.

  47. Ungprasert P, Sanguankeo A, Upala S, Knight EL. Risk of malignancy in patients with giant cell arteritis and polymyalgia rheumatica: A systematic review and meta-analysis. Semin Arthritis Rheum 2014 Jun 26. doi: 10.1016/j.semarthrit.2014.06.004. Overall, the risk of malignancy in GCA and PMR is not higher than in the general population.

  48. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Gomez-Acebo I, Pego-Reigosa R, Lopez-Diaz MJ, Vazquez-Triñanes MC, et al. Strokes at time of disease diagnosis in a series of 287 patients with biopsy-proven giant cell arteritis. Medicine (Baltimore). 2009;88:227–35.

  49. Tomasson G, Peloquin C, Mohammad A, Love TJ, Zhang Y, Choi HK, et al. Risk for cardiovascular disease early and late after a diagnosis of giant-cell arteritis: a cohort study. Ann Intern Med. 2014;160:73–80. Unlike previous studies, a recent report suggests that cardiovascular disease may be increased in GCA.

  50. Schmidt J, Kermani TA, Muratore F, Crowson CS, Matteson EL, Warrington KJ. Statin use in giant cell arteritis: a retrospective study. J Rheumatol. 2013;40:910–5. The use of statins may reduce the risk of GCA.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

Download references

Compliance with Ethics Guidelines

Conflict of Interest

Miguel A. González-Gay and Trinitario Pina declare no conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

  1. Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Avenida de Valdecilla, s/n, 39008, Santander, Spain

    Miguel A. González-Gay & Trinitario Pina

  2. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa

    Miguel A. González-Gay

Authors
  1. Miguel A. González-Gay
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Trinitario Pina
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Miguel A. González-Gay.

Additional information

This article is a part of the Topical Collection on Vasculitis

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

González-Gay, M.A., Pina, T. Giant Cell Arteritis and Polymyalgia Rheumatica: an Update. Curr Rheumatol Rep 17, 6 (2015). https://doi.org/10.1007/s11926-014-0480-1

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11926-014-0480-1

Keywords

Search

Navigation