How the ultrasound protocol may improve the timely diagnosis of cerebrovascular complications in giant cell arteritis
Abstract
Giant cell arteritis (GCA) is a granulomatous inflammatory vasculitis of medium and large vessels, with a predilection for the external carotid and ophthalmic arteries and, to a lesser extent, for the vertebral arteries. In early phases of the disease, symptoms may be nonspecific, such as malaise, fever, and weight loss. Overt typical GCA symptoms are temporal headache, scalp tenderness, jaw claudication, and sudden vision loss. Inflammatory vessel involvement in GCA results in partial or complete occlusion of the arterial lumen, leading to complications such as acute ischemic optic neuropathy, transient ischemic attack, and ischemic stroke. The latter is a rare but severe complication of GCA, and it has been reported in 2.8-7% of patients diagnosed with GCA. The majority of ischemic strokes are related to inflammation of vertebral and, less frequently, basilar and internal carotid arteries. Stroke in GCA patients affects vertebrobasilar circulation in 50 to 100% of cases, compared to only 20% observed in cerebrovascular accidents in the general population. Prompt diagnosis of GCA cranial involvement is pivotal, since early start of high-dose corticosteroid treatment and/or immunosuppressive drugs (e.g., tocilizumab and methotrexate) is highly effective in preventing further evolution and recurrence of such complications. In this viewpoint, we have briefly pinpointed the current possible value of vertebral ultrasound from both the rheumatologist’s and neurologist’s points of view.
Keywords
References
- Soriano A, Muratore F, Pipitone N, Boiardi L, Cimino L, Salvarani C. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol 2017; 13: 476-84. DOI: https://doi.org/10.1038/nrrheum.2017.98
- Fein AS, Ko MW. Neuro-ophthalmologic complications of giant cell arteritis: diagnosis and treatment. Semin Neurol 2019; 39: 673-81. DOI: https://doi.org/10.1055/s-0039-1698761
- Bilton EJ, Mollan SP. Giant cell arteritis: reviewing the advancing diagnostics and management. Eye 2023; 37: 2365-73. DOI: https://doi.org/10.1038/s41433-023-02433-y
- Coronel L, Rodríguez-Pardo J, Monjo I, de Miguel E. Prevalence and significance of ischemic cerebrovascular events in giant cell arteritis. Med Clin 2021; 157: 53-7. DOI: https://doi.org/10.1016/j.medcli.2020.05.068
- Pariente A, Guédon A, Alamowitch S, Thietart S, Carrat F, Delorme S, et al. Ischemic stroke in giant-cell arteritis: French retrospective study. J Autoimmun 2019; 99: 48-51. DOI: https://doi.org/10.1016/j.jaut.2019.01.009
- Marte Furment M, Antigua Jimenez S, Pabolu S. Acute stroke due to vertebral artery dissection in giant cell arteritis. Case Rep Rheumatol 2021; 2021: 5518541. DOI: https://doi.org/10.1155/2021/5518541
- Bajko Z, Balasa R, Maier S, Motataianu A, Barcutean L, Andone S, et al. Stroke secondary to giant-cell arteritis: a literature review. Exp Ther Med 2021; 22: 876. DOI: https://doi.org/10.3892/etm.2021.10308
- Wiszniewska M, Devuyst G, Bogousslavsky J. Giant cell arteritis as a cause of first-ever stroke. Cerebrovasc Dis 2007; 24: 226-30. DOI: https://doi.org/10.1159/000104482
- Monti S, Floris A, Ponte CB, Schmidt WA, Diamantopoulos AP, Pereira C, et al. The proposed role of ultrasound in the management of giant cell arteritis in routine clinical practice. Rheumatology 2018; 57: 112-9. DOI: https://doi.org/10.1093/rheumatology/kex341
- Dejaco C, Ramiro S, Bond M, Bosch P, Ponte C, Mackie SL, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update. Ann Rheum Dis 2024; 83: 741-51. DOI: https://doi.org/10.1136/ard-2023-224543
- Mukhtyar CB, Beadsmoore C, Ducker G, Fordham S, Sisson K, Jones C. Ultrasonography led multimodal diagnostic pathway for giant cell arteritis. Rheumatology 2025; 64: 2077-82. DOI: https://doi.org/10.1093/rheumatology/keae493
- Bosch P, Bond M, Dejaco C, Ponte C, Mackie SL, Falzon L, et al. Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations. RMD Open 2023; 9: e003379. DOI: https://doi.org/10.1136/rmdopen-2023-003379
- van Nieuwland M, Colin EM, Vermeer M, Wagenaar NRL, Vijlbrief OD, van Zandwijk JK, et al. A direct comparison in diagnostic performance of CDUS, FDG-PET/CT and MRI in patients suspected of giant cell arteritis. Rheumatology 2025; 64: 1392-9. DOI: https://doi.org/10.1093/rheumatology/keae171
- Skoog J, Svensson C, Eriksson P, Sjöwall C, Zachrisson H. The diagnostic performance of an extended ultrasound protocol in patients with clinically suspected giant cell arteritis. Front Med 2021; 8: 807996. DOI: https://doi.org/10.3389/fmed.2021.807996
- Soares C, Costa A, Santos R, Abreu P, Castro P, Azevedo E. Clinical, laboratory and ultrasonographic interrelations in giant cell arteritis. J Stroke Cerebrovasc Dis 2021; 30: 105601. DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2021.105601
- Kargiotis O, Psychogios K, Safouris A, Bakola E, Andreadou E, Karapanayiotides T, et al. Cervical duplex ultrasound for the diagnosis of giant cell arteritis with vertebral artery involvement. J Neuroimaging 2021; 31: 656-64. DOI: https://doi.org/10.1111/jon.12857
- Lambrechts RA, Uyttenboogaart M, Drost G. A vertebral artery halo sign indicates giant cell arteritis affecting the posterior circulation of the brain. Lancet 2021; 397: e6. DOI: https://doi.org/10.1016/S0140-6736(21)00248-8
- Penet T, Lambert M, Baillet C, Outteryck O, Hénon H, Morell-Dubois S, et al. Giant cell arteritis-related cerebrovascular ischemic events: a French retrospective study of 271 patients, systematic review of the literature and meta-analysis. Arthritis Res Ther 2023; 25: 116. DOI: https://doi.org/10.1186/s13075-023-03091-x
- García-García J, Ayo-Martín Ó, Argandoña-Palacios L, Segura T. Vertebral artery halo sign in patients with stroke: a key clue for the prompt diagnosis of giant cell arteritis. Stroke 2011; 42: 3287-90. DOI: https://doi.org/10.1161/STROKEAHA.111.625152
- Psychogios K, Magoufis G, Kargiotis O, Safouris A, Bakola E, Chondrogianni M, et al. Ultrasound assessment of extracranial carotids and vertebral arteries in acute cerebral ischemia. Medicina 2020; 56: 711. DOI: https://doi.org/10.3390/medicina56120711
- Reinhard M, Schmidt D, Schumacher M, Hetzel A. Involvement of the vertebral arteries in giant cell arteritis mimicking vertebral dissection. J Neurol 2003; 250: 1006-9. DOI: https://doi.org/10.1007/s00415-003-1155-4
- de Bray JM, Penisson-Besnier I, Dubas F, Emile J. Extracranial and intracranial vertebrobasilar dissections: diagnosis and prognosis. J Neurol Neurosurg Psychiatry 1997; 63: 46-51. DOI: https://doi.org/10.1136/jnnp.63.1.46
- Ben Hassen W, Machet A, Edjlali-Goujon M, Legrand L, Ladoux A, Mellerio C, et al. Imaging of cervical artery dissection. Diagn Interv Imaging 2014; 95: 1151-61. DOI: https://doi.org/10.1016/j.diii.2014.10.003
- Zwicker J, Atkins EJ, Lum C, Sharma M. An atypical presentation of giant cell arteritis. CMAJ 2011; 183: E301-5. DOI: https://doi.org/10.1503/cmaj.100380
- Rüegg S, Engelter S, Jeanneret C, Hetzel A, Probst A, Steck AJ, et al. Bilateral vertebral artery occlusion resulting from giant cell arteritis: report of 3 cases and review of the literature. Medicine 2003; 82: 1-12. DOI: https://doi.org/10.1097/00005792-200301000-00001
- Bajko Z, Motataianu A, Stoian A, Barcutean L, Andone S, Maier S, et al. Prevalence and clinical characteristics of subclavian steal phenomenon/syndrome in patients with acute ischemic stroke. J Clin Med 2021; 10: 5237. DOI: https://doi.org/10.3390/jcm10225237
- Zalaquett Z, Zgheib G, Karam L, Zaatar M, Nehme K, Tabet G. A rare association of subclavian steal syndrome with bilateral carotid artery stenosis: a case report. J Vasc Surg Cases Innov Tech 2023; 9: 101243. DOI: https://doi.org/10.1016/j.jvscit.2023.101243
- De Brucker Y, Peeters E, Goethals LR, Van Looveren K, François O, Van Hedent E. The vertebral bunny waveform: an early manifestation of the subclavian steal syndrome. JBR-BTR 2012; 95: 340. DOI: https://doi.org/10.5334/jbr-btr.692
- Mahjoub Y, McKenzie ED, Raouf S, Almekhlafi M. Progressive vertebrobasilar vasculopathy and stroke secondary to giant cell arteritis. Stroke 2022; 53: e435-8. DOI: https://doi.org/10.1161/STROKEAHA.122.039503
- Smith AT, Wilbert CD, Ferre RM. Using the retrobulbar spot sign to assist in diagnosis and management of central retinal artery occlusions. J Ultrasound Med 2020; 39: 197-202. DOI: https://doi.org/10.1002/jum.15073
- Sebastian A, Tomelleri A, Kayani A, Prieto-Pena D, Ranasinghe C, Dasgupta B. Probability-based algorithm using ultrasound and additional tests for suspected GCA in a fast-track clinic. RMD Open 2020; 6: e001297. DOI: https://doi.org/10.1136/rmdopen-2020-001297
- Sebastian A, van der Geest KSM, Tomelleri A, Macchioni P, Klinowski G, Salvarani C, et al. Development of a diagnostic prediction model for giant cell arteritis by sequential application of Southend giant cell arteritis probability score and ultrasonography: a prospective multicentre study. Lancet Rheumatol 2024; 6: e291-9. DOI: https://doi.org/10.1016/S2665-9913(24)00027-4
- Prünte MKR, Naumann A, Christ M, Naumann M, Bayas A. Giant cell arteritis with vertebral artery involvement-baseline characteristics and follow-up of a monocentric patient cohort. Front Neurol 2023; 14: 1188073. DOI: https://doi.org/10.3389/fneur.2023.1188073
- Bonnan M, Debeugny S. Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy? Front Neurol 2023; 14: 1305093. DOI: https://doi.org/10.3389/fneur.2023.1305093
- de Boysson H, Liozon E, Larivière D, Samson M, Parienti JJ, Boutemy J, et al. Giant cell arteritis-related stroke: a retrospective multicenter case-control study. J Rheumatol 2017; 44: 297-303. DOI: https://doi.org/10.3899/jrheum.161033
- 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 2009; 88: 227-35. DOI: https://doi.org/10.1097/MD.0b013e3181af4518
- González-Gay MA, Blanco R, Rodríguez-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. DOI: https://doi.org/10.1002/1529-0131(199808)41:8<1497::AID-ART22>3.0.CO;2-Z
- Hočevar A, Ješe R, Tomšič M, Rotar Ž. Risk factors for severe cranial ischaemic complications in giant cell arteritis. Rheumatology 2020; 59: 2953-9. DOI: https://doi.org/10.1093/rheumatology/keaa058
- Parreau S, Dumonteil S, Montoro FM, Gondran G, Bezanahary H, Palat S, et al. Giant cell arteritis-related stroke in a large inception cohort: a comparative study. Semin Arthritis Rheum 2022; 55: 152020. DOI: https://doi.org/10.1016/j.semarthrit.2022.152020
- Lo Gullo A, Koster MJ, Crowson CS, Makol A, Ytterberg SR, Saitta A, et al. Venous thromboembolism and cerebrovascular events in patients with giant cell arteritis: a population-based retrospective cohort study. PLoS One 2016; 11: e0149579. DOI: https://doi.org/10.1371/journal.pone.0149579
- Guggenberger KV, Vogt ML, Song JW, Fröhlich M, Schmalzing M, Venhoff N, et al. High-resolution magnetic resonance imaging visualizes intracranial large artery involvement in giant cell arteritis. Rheumatology 2025; 64: 842-8. DOI: https://doi.org/10.1093/rheumatology/keae010
Metrics
Citations and online attention from Dimensions, Altmetric and PlumX.
License
Copyright (c) 2025 the Author(s)

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.