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Clinical features of polymyalgia rheumatica and giant cell arteritis

Abstract

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory diseases that typically affect white individuals >50 years. Women are affected 2–3 times more often than men. PMR and GCA occur together more frequently than expected by chance. The main symptoms of PMR are pain and stiffness in the shoulders, and often in the neck and pelvic girdle. Imaging studies reveal inflammation of joints and bursae of the affected areas. GCA is a large-vessel and medium-vessel arteritis predominantly involving the branches of the aortic arch. The typical clinical manifestations of GCA are new headache, jaw claudication and visual loss. PMR and GCA usually remit within 6 months to 2 years from disease onset. Some patients, however, have a relapsing course and might require long-standing treatment. Diagnosis of PMR and GCA is based on clinical features and elevated levels of inflammatory markers. Temporal artery biopsy remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement in GCA. Glucocorticoids remain the cornerstone of treatment of both PMR and GCA, but patients with GCA require higher doses. Synthetic immunosuppressive drugs also have a role in disease management, whereas the role of biologic agents is currently unclear.

Key Points

  • Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are linked disorders that predominantly affect elderly white individuals

  • The diagnosis of GCA and PMR is made on the basis of characteristic clinical features and elevated inflammatory markers, but temporal artery biopsy remains the diagnostic gold standard for GCA

  • Early diagnosis is necessary to prevent ischaemic events in GCA; patients with GCA who suffer an ischaemic event are at high risk of developing another attack unless immediately treated

  • Imaging studies are required to diagnose and monitor GCA with large-vessel involvement

  • Glucocorticoids remain the mainstay of treatment for PMR and GCA

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Figure 1: Pathogenetic mechanisms operating in GCA.
Figure 2: Typical distribution of pain in patients with PMR.
Figure 3: Ultrasonographical findings for GCA.
Figure 4: Early diagnosis of GCA using PET.
Figure 5: Imaging evidence for bursitis in patients with PMR.

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C. Salvarani and N. Pipitone researched data for the article. C. Salvarani, N. Pipitone and A. Versari made equal contributions to discussion of content. N. Pipitone wrote the article. All authors contributed equally to reviewing and editing the manuscript before submission.

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Correspondence to Carlo Salvarani.

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Salvarani, C., Pipitone, N., Versari, A. et al. Clinical features of polymyalgia rheumatica and giant cell arteritis. Nat Rev Rheumatol 8, 509–521 (2012). https://doi.org/10.1038/nrrheum.2012.97

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