Giant Cell Arteritis (GCA)
GCA occurs primarily in patients >50 years of age. The incidence increases with age, and GCA is almost 10 times more common among patients in their 80s than in patients aged 50 to 60 years. GCA is two to three times more common among women than men.
Most patients will have one of five presentations:
Some 20% of patients have cranial symptoms with superficial headache, scalp tenderness, jaw and tongue claudication, and rarely scalp necrosis, diplopia, or blindness.
Approximately 40% of patients have polymyalgia rheumatica (PMR) with pain and stiffness of proximal muscle groups, such as the neck, shoulders, hips, and thighs. Muscle symptoms are usually symmetric and the shoulders are most commonly involved. Note that 10% to 20% of patients presenting with PMR develop GCA on follow-up.
Some 20% of patients have both cranial and PMR symptoms. About 40% to 50% of patients with GCA have PMR symptoms.
About 15% of patients have fever and systemic symptoms without any localized symptoms. Patients can present with a fever of unknown origin (FUO). GCA is the cause in 15% of elderly patients with an FUO.
Some 5% of patients have other symptoms, such as cough, claudication (upper > lower extremity), or synovitis.
-->GCA should be suspected in individuals aged >50 to 60 years with new onset of unexplained pain above the neck (headache, jaw claudication, other areas mimicking sinusitis/ear pain), unexplained fever, or PMR.
GCA facts and clinic
- Large vessel vasculitis, especially of the thoracic aorta and its branches
- polymyalgic pain shoulder/hip girdle, neck stiffness
- Increased CRP/increased BSR
- Peripheral arthritis/RS3PE syndrome
- Systemic symptoms (fever, weight loss (>2 kg), night sweats, depression) Headache
- Sensitive/painful scalp Vascular swelling/pain, auscultation sound Claudicatio masticatoris/pain and claudication of the tongue
- Blurred vision: Amaurosis fugax, double vision, blindness
- (specific findings: AION, central ocular muscle paralysis, occlusion of arterial retinal vessels and/or choroidal ischemia)
- difficulty swallowing / dysphagia
- ischemia of the extremities, missing or asymmetric pulses, blood pressure difference, Raynaud's phenomenon
- Aortic Insufficiency
Complications for Different Vascular Distributions in Giant Cell Arteritis:
There have been many instances that support the hypothesis that corticosteroid therapy can prevent catastrophic events in patients with temporal arteritis. Sudden blindness and other stroke-like events have occasionally been reversed in 33% of patients by institution of high-dose corticosteroid therapy (1 g of intravenous methylprednisolone daily for 3 days) if started within 24 hours of the event. Consider Tocilizumab-therapy.
The risk of death from GCA appears to be increased (threefold) within the first 4 months of starting therapy. Patients typically die of vascular complications, such as stroke or myocardial infarction, supporting the use of low-dose aspirin in patients diagnosed with GCA. BP must be controlled and the patient should stop smoking.
Surgery is considered when the aneurysm enlarges to greater than 5 cm or dissects.
Dejaco C, Brouwer E, Mason JC, et al.: Giant cell arteritis and polymyalgia rheumatica: current challenges and opportu- nities. Nat Rev Rheumatol 2017; 13: 578–592.
Cimmino MA, Camellino D: Large vessel vasculitis: is it more common than usually assumed? Reumatismo 2017; 69: 143– 146.
Buttgereit F, Dejaco C, Matteson EL, Dasgupta B: Polymyalgia rheumatica and giant cell arteritis: a systematic review. JAMA 2016; 315: 2442–2458.
Camellino D, Giusti A, Girasole G, Bianchi G, Dejaco C: Patho- genesis, diagnosis and management of polymyalgia rheuma- tica. Drugs Aging 2019; 36: 1015–1026.
van der Geest KS, Abdulahad WH, Chalan P, et al.: Disturbed B cell homeostasis in newly diagnosed giant cell arteritis and polymyalgia rheumatica. Arthritis Rheumatol 2014; 66: 1927– 1938.
Straub RH, Glück T, Cutolo M, et al.: The adrenal steroid status in relation to inflammatory cytokines (interleukin-6 and tumour necrosis factor) in polymyalgia rheumatica. Rheumatology (Ox- ford) 2000; 39: 624–631.
Calabrese C, Cappelli LC, Kostine M, Kirchner E, Braaten T, Calabrese L: Polymyalgia rheumatica-like syndrome from checkpoint inhibitor therapy: case series and systematic review of the literature. RMD Open 2019; 5: e000906.
Hellmich B, Agueda A, Monti S, et al.: 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2020; 79: 19–30.
Buttgereit F, DGRh, Brabant T, et al.: [S3 guidelines on treat- ment of polymyalgia rheumatica : Evidence-based guidelines of the German Society of Rheumatology (DGRh), the Austrian Society of Rheumatology and Rehabilitation (OGR) and the Swiss Society of Rheumatology (SGT) and participating medi- cal scientific specialist societies and other organizations]. Z Rheumatol 2018; 77: 429–441.
Matteson EL, Dejaco C: Polymyalgia rheumatica. Ann Intern Med 2017; 166: ITC65-ITC80.
Dejaco C, Singh YP, Perel P, et al.: Recommendations for the management of polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology col- laborative initiative. Ann Rheum Dis 2015; 74: 1799–1807.
Schönau V, Vogel K, Englbrecht M, et al.: The value of (18)F- FDG-PET/CT in identifying the cause of fever of unknown origin (FUO) and inflammation of unknown origin (IUO): data from a prospective study. Ann Rheum Dis 2018; 77: 70–77.
Kermani TA, Diab S, Sreih AG, et al.: Arterial lesions in giant cell arteritis: A longitudinal study. Semin Arthritis Rheum 2019; 48: 707–713.
Hernandez-Rodriguez J, Murgia G, Villar I, et al.: Description and validation of histological patterns and proposal of a dynamic model of inflammatory infiltration in giant-cell arte- ritis. Medicine (Baltimore) 2016; 95: e2368.
Koster MJ, Warrington KJ: Giant cell arteritis: pathogenic mechanisms and new potential therapeutic targets. BMC Rheumatol 2017; 1: 2.
Boland P, Heath J, Sandigursky S: Immune checkpoint inhi- bitors and vasculitis. Curr Opin Rheumatol 2020; 32: 53–56.
Langford CA, Cuthbertson D, Ytterberg SR, et al.: A Rand- omized, double-blind trial of abatacept (CTLA-4Ig) for the treatment of giant cell arteritis. Arthritis Rheumatol 2017; 69: 837–845.
18. Stone JH, Tuckwell K, Dimonaco S, et al.: Trial of tocilizumab in giant-cell arteritis. N Engl J Med 2017; 377: 317–328.
19. Chan FLY, Lester S, Whittle SL, Hill CL: The utility of ESR, CRP and platelets in the diagnosis of GCA. BMC Rheumatol 2019; 3: 14.
20. Diamantopoulos AP, Haugeberg G, Lindland A, Myklebust G: The fast-track ultrasound clinic for early diagnosis of giant cell arteritis significantly reduces permanent visual impair- ment: towards a more effective strategy to improve clinical outcome in giant cell arteritis? Rheumatology (Oxford) 2016; 55:66–70.
21. Dejaco C, Ramiro S, Duftner C, et al.: EULAR recommendations for the use of imaging in large vessel vasculitis in clinical prac- tice. Ann Rheum Dis 2018; 77: 636–643.
22. Achkar AA, et al.: How does previous corticosteroid treatment affect the biopsy findings in giant cell (temporal) arteritis? Ann Intern Med 1994; 120: 987–992.
23. Berger CT, Sommer G, Aschwanden M, Staub D, Rottenburger C, Daikeler T: The clinical benefit of imaging in the diagnosis and treatment of giant cell arteritis. Swiss Med Wkly 2018; 148: w14661.
24. Reichenbach S, Adler S, Bonel H, et al.: Magnetic resonance angiography in giant cell arteritis: results of a randomized controlled trial of tocilizumab in giant cell arteritis. Rheuma- tology (Oxford) 2018; 57: 982–986.
25. Muratore F, Kermani TA, Crowson CS, et al.: Large-vessel dila- tation in giant cell arteritis: a different subset of disease? Arthritis Care Res (Hoboken) 2018; 70: 1406–1411.
26. Buttgereit F, Matteson EL, Dejaco C, Dasgupta B: Prevention of glucocorticoid morbidity in giant cell arteritis. Rheumatology (Oxford) 2018; 57: ii11–ii21.
27. Hocevar A, Ješe R, Rotar Ž, Tomšič M.: Does leflunomide have a role in giant cell arteritis? An open-label study. Clin Rheumatol 2019; 38: 291–296.
28. Tengesdal S, Diamantopoulos AP, Myklebust G: Leflunomide versus methotrexate in treatment of giant cell arteritis: com- parison of efficacy, safety, and drug survival. Scand J Rheuma- tol. 2019; 48: 333–335.
29. Gloor AD, Yerly D, Adler S, et al.: Immuno-monitoring reveals an extended subclinical disease activity in tocilizumab-treated giant cell arteritis. Rheumatology (Oxford) 2018; 57: 1795– 1801.
30. Adler S, Reichenbach S, Gloor A, Yerly D, Cullmann JL, Villiger PM: Risk of relapse after discontinuation of tocilizumab thera- py in giant cell arteritis. Rheumatology (Oxford) 2019; 58: 1639–1643.
31. Nielsen BD, Gormsen LC, Hansen IT, Keller KK, Therkildsen P, Hauge EM: Three days of high-dose glucocorticoid treatment attenuates large-vessel 18F-FDG uptake in large-vessel giant cell arteritis but with a limited impact on diagnostic accuracy. Eur J Nucl Med Mol Imaging 2018; 45: 1119–1128.