Skip to main content
Log in

Orbital magnetic resonance imaging of giant cell arteritis with ocular manifestations: a systematic review and individual participant data meta-analysis

  • Head and Neck
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Objectives

We conducted a systematic review and individual participant data meta-analysis of publications reporting the ophthalmologic presentation, clinical exam, and orbital MRI findings in patients with giant cell arteritis and ocular manifestations.

Methods

PubMed and Cochrane databases were searched up to January 16, 2022. Publications reporting patient-level data on patients with ophthalmologic symptoms, imaged with orbital MRI, and diagnosed with biopsy-proven giant cell arteritis were included. Demographics, clinical symptoms, exam, lab, imaging, and outcomes data were extracted. The methodological quality and completeness of reporting of case reports were assessed.

Results

Thirty-two studies were included comprising 51 patients (females = 24; median age, 76 years). Vision loss (78%) and headache (45%) were commonly reported visual and cranial symptoms. Ophthalmologic presentation was unilateral (41%) or bilateral (59%). Fundus examination most commonly showed disc edema (64%) and pallor (49%). Average visual acuity was very poor (2.28 logMAR ± 2.18). Diagnoses included anterior (61%) and posterior (16%) ischemic optic neuropathy, central retinal artery occlusion (8%), and orbital infarction syndrome (2%). On MRI, enhancement of the optic nerve sheath (53%), intraconal fat (25%), and optic nerve/chiasm (14%) was most prevalent. Among patients with monocular visual symptoms, 38% showed pathologic enhancement in the asymptomatic orbit. Six of seven cases reported imaging resolution after treatment on follow-up MRIs.

Conclusions

Vision loss, pallid disc edema, and optic nerve sheath enhancement are the most common clinical, fundoscopic, and imaging findings reported in patients diagnosed with giant cell arteritis with ocular manifestations, respectively. MRI may detect subclinical inflammation and ischemia in the asymptomatic eye and may be an adjunct diagnostic tool.

Clinical relevance statement

Brain and orbital MRIs may have diagnostic and prognostic roles in patients with suspected giant cell arteritis who present with ophthalmic symptoms.

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
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

Abbreviations

BB-MRI:

Black-blood MRI

GCA:

Giant cell arteritis

TAB:

Temporal artery biopsy

VA:

Visual acuity

References

  1. Hayreh SS, Podhajsky PA, Zimmerman B (1998) Ocular manifestations of giant cell arteritis. Am J Ophthalmol 125:509–520

    CAS  PubMed  Google Scholar 

  2. Salvarani C, Gabriel SE, O’Fallon WM, Hunder GG (1995) The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern. Ann Intern Med 123:192–194

    CAS  PubMed  Google Scholar 

  3. Chandran AK, Udayakumar PD, Crowson CS, Warrington KJ, Matteson EL (2015) The incidence of giant cell arteritis in Olmsted County, Minnesota, over a 60-year period 1950–2009. Scand J Rheumatol 44:215–218

    CAS  PubMed  PubMed Central  Google Scholar 

  4. Smith CA, Fidler WJ, Pinals RS (1983) The epidemiology of giant cell arteritis. Report of a ten-year study in Shelby County. Arthritis Rheum 26:1214–1219

    CAS  PubMed  Google Scholar 

  5. Salvarani C, Crowson CS, O’Fallon WM, Hunder GG, Gabriel SE (2004) Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period. Arthritis Rheum 51:264–268

    PubMed  Google Scholar 

  6. Saleh M, Turesson C, Englund M, Merkel PA, Mohammad AJ (2016) Visual complications in patients with biopsy-proven giant cell arteritis: a population-based study. J Rheumatol 43:1559–1565

    PubMed  PubMed Central  Google Scholar 

  7. Ji J, Dimitrijevic I, Sundquist J, Sundquist K, Zöller B (2017) Risk of ocular manifestations in patients with giant cell arteritis: a nationwide study in Sweden. Scand J Rheumatol 46:484–489

    CAS  PubMed  Google Scholar 

  8. González-Gay MA, García-Porrúa C, Llorca J et al (2000) Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. Medicine (Baltimore) 79:283–292

    PubMed  Google Scholar 

  9. Costello F, Zimmerman MB, Podhajsky PA, Hayreh SS (2004) Role of thrombocytosis in diagnosis of giant cell arteritis and differentiation of arteritic from non-arteritic anterior ischemic optic neuropathy. Eur J Ophthalmol 14:245–257

    CAS  PubMed  Google Scholar 

  10. D’Souza NM, Morgan ML, Almarzouqi SJ, Lee AG (2016) Magnetic resonance imaging findings in giant cell arteritis. Eye (Lond) 30:758–762

    PubMed  Google Scholar 

  11. Berger CT, Sommer G, Aschwanden M, Staub D, Rottenburger C, Daikeler T (2018) The clinical benefit of imaging in the diagnosis and treatment of giant cell arteritis. Swiss Med Wkly 148:w14661

    PubMed  Google Scholar 

  12. Bley TA, Wieben O, Uhl M, Thiel J, Schmidt D, Langer M (2005) High-resolution MRI in giant cell arteritis: imaging of the wall of the superficial temporal artery. AJR Am J Roentgenol 184:283–287

    PubMed  Google Scholar 

  13. Mohammed-Brahim N, Clavel G, Charbonneau F et al (2019) Three Tesla 3D high-resolution vessel wall MRI of the orbit may differentiate arteritic from nonarteritic anterior ischemic optic neuropathy. Invest Radiol 54:712–718

    PubMed  Google Scholar 

  14. Guggenberger KV, Vogt ML, Song JW et al (2022) Intraorbital findings in giant cell arteritis on black blood MRI. Eur Radiol. https://doi.org/10.1007/s00330-022-09256-7

    Article  PubMed  PubMed Central  Google Scholar 

  15. AlShaker S, Shemesh AA, Margolin E (2018) Enhancement of optic nerve sheath in AAION: a case of visual recovery in fulminant GCA. Can J Ophthalmol 53:e236–e239

    PubMed  Google Scholar 

  16. Lee AG, Eggenberger ER, Kaufman DI, Manrique C (1999) Optic nerve enhancement on magnetic resonance imaging in arteritic ischemic optic neuropathy. J Neuroophthalmol 19:235–237

    CAS  PubMed  Google Scholar 

  17. Nambiema A, Sembajwe G, Lam J et al (2021) A protocol for the use of case reports/studies and case series in systematic reviews for clinical toxicology. Front Med (Lausanne) 8:708380

    PubMed  Google Scholar 

  18. Tierney JF SL, Clarke M (2022) Chapter 26: individual partcipant data. In: Higgins JPT TJ, Chandler J, Cumpston M, Li T, Page MJ, Welch VA (editors), (ed) Cochrane Handbook for Systematic Reviews of Interventions version 63. Cochrane

  19. Gagnier JJ, Kienle G, Altman DG, Moher D, Sox H, Riley D (2014) The CARE guidelines: consensus-based clinical case report guideline development. J Clin Epidemiol 67:46–51

    PubMed  Google Scholar 

  20. Institute JB (2017) Checklist for Case Reports. The Joana Briggs Institute, www.joannabriggs.org. Available via https://jbi.global/sites/default/files/2019-05/JBI_Critical_Appraisal-Checklist_for_Case_Reports2017_0.pdf. Accessed November 10, 2022 2022

  21. Holladay JT (2004) Visual acuity measurements. J Cataract Refract Surg 30:287–290

    PubMed  Google Scholar 

  22. Kniestedt C, Stamper RL (2003) Visual acuity and its measurement. Ophthalmol Clin North Am 16:155–170

    PubMed  Google Scholar 

  23. Virgili G, Acosta R, Bentley SA, Giacomelli G, Allcock C, Evans JR (2018) Reading aids for adults with low vision. Cochrane Database Syst Rev 4:Cd003303

  24. Kim DD, Budhram A, Pelz D, MacDonald M (2016) Teaching neuroimages: orbital infarction syndrome from giant cell arteritis. Neurology 87:e269

    PubMed  Google Scholar 

  25. Lee AG, Tang RA, Feldon SE et al (2001) Orbital presentations of giant cell arteritis. Graefes Arch Clin Exp Ophthalmol 239:509–513

    CAS  PubMed  Google Scholar 

  26. Gospe SM 3rd, Amrhein TJ, Malinzak MD, Bhatti MT, Mettu P, El-Dairi MA (2021) Magnetic resonance imaging abnormalities of the optic nerve sheath and intracranial internal carotid artery in giant cell arteritis. J Neuroophthalmol 41:54–59

    PubMed  Google Scholar 

  27. Liu KC, Chesnutt DA (2013) Perineural optic nerve enhancement on magnetic resonance imaging in giant cell arteritis. J Neuroophthalmol 33:279–281

    PubMed  Google Scholar 

  28. Emami S, Howarth D, Margolin E (2021) Giant cell arteritis presenting as bilateral optic perineuritis in an african man. J Neuroophthalmol 41:e149–e152

    PubMed  Google Scholar 

  29. Tashiro T, Tsujimoto A, Nakamura N (2021) Ischemic optic neuropathy despite pulse methylprednisolone therapy in a giant cell arteritis patient with perineural optic nerve enhancement. Rinsho Shinkeigaku 61:851–855

    PubMed  Google Scholar 

  30. Geiger J, Ness T, Uhl M et al (2009) Involvement of the ophthalmic artery in giant cell arteritis visualized by 3T MRI. Rheumatology (Oxford) 48:537–541

    PubMed  Google Scholar 

  31. Ross M, Bursztyn L, Superstein R, Gans M (2017) Multiple cranial nerve palsies in giant cell arteritis. J Neuroophthalmol 37:398–400

    PubMed  Google Scholar 

  32. Albarrak AM, Mohammad Y, Hussain S, Husain S, Muayqil T (2018) Simultaneous bilateral posterior ischemic optic neuropathy secondary to giant cell arteritis: a case presentation and review of the literature. BMC Ophthalmol 18:317

    PubMed  PubMed Central  Google Scholar 

  33. Biotti D, Savatovski J, Bidot S et al (2011) Diffusion MR imaging of the optic nerve in a patient with giant-cell arteritis and vertebral artery vasculitis. Eur Neurol 66:83–84

    CAS  PubMed  Google Scholar 

  34. Garcia-Porrua C, Santamarina R, Armesto V, Gonzalez-Gay MA (2005) Magnetic resonance imaging in optic neuritis due to giant cell arteritis. Arthritis Rheum 53:313–314

    PubMed  Google Scholar 

  35. Heraud A, Zmuda M, Lecler A (2018) Lacrimal gland ischemia due to giant cell arteritis. Ophthalmology 125:1233

    PubMed  Google Scholar 

  36. Garcia-Porrua C, Pego-Reigosa R, Armesto V, Gonzalez-Gay MA (2003) Neovascularization around the optic nerve in giant cell arteritis. Arthritis Rheum 49:737–738

    PubMed  Google Scholar 

  37. Kornberg MD, Ratchford JN, Subramaniam RM, Probasco JC (2015) Giant cell arteritis mimicking infiltrative leptomeningeal disease of the optic nerves. BMJ Case Rep 2015

  38. Donaldson L, Nanji K, Rebello R, Khalidi NA, Rodriguez AR (2020) Involvement of the intracranial circulation in giant cell arteritis. Can J Ophthalmol 55:391–400

    PubMed  Google Scholar 

  39. Hittinger M, Berlis A, Pfadenhauer K (2015) Inflammatory Pseudo-tumour Orbitae (PTO): an atypical manifestation of giant cell arteritis (GCA). Clin Neuroradiol 25:411–414

    CAS  PubMed  Google Scholar 

  40. Morgenstern KE, Ellis BD, Schochet SS, Linberg JV (2003) Bilateral optic nerve sheath enhancement from giant cell arteritis. J Rheumatol 30:625–627

    PubMed  Google Scholar 

  41. Joelson E, Ruthrauff B, Ali F, Lindeman N, Sharp FR (2000) Multifocal dural enhancement associated with temporal arteritis. Arch Neurol 57:119–122

    CAS  PubMed  Google Scholar 

  42. Nassani S, Cocito L, Arcuri T, Favale E (1995) Orbital pseudotumor as a presenting sign of temporal arteritis. Clin Exp Rheumatol 13:367–369

    CAS  PubMed  Google Scholar 

  43. Choi JH, Shin JH, Jung JH (2019) Arteritic anterior ischemic optic neuropathy associated with giant-cell arteritis in Korean patients: a retrospective single-center analysis and review of the literature. J Clin Neurol 15:386–392

    PubMed  PubMed Central  Google Scholar 

  44. Hayreh SS (2021) Giant cell arteritis: its ophthalmic manifestations. Indian J Ophthalmol 69:227–235

    PubMed  PubMed Central  Google Scholar 

  45. Killer HE, Laeng HR, Flammer J, Groscurth P (2003) Architecture of arachnoid trabeculae, pillars, and septa in the subarachnoid space of the human optic nerve: anatomy and clinical considerations. Br J Ophthalmol 87:777–781

    CAS  PubMed  PubMed Central  Google Scholar 

  46. Hayreh SS (1991) Ophthalmic features of giant cell arteritis. Baillieres Clin Rheumatol 5:431–459

    CAS  PubMed  Google Scholar 

  47. Zhang K-J, Li M-X, Zhang P, Qin H-Q, Guo Z-N, Yang Y (2022) Validity of high resolution magnetic resonance imaging in detecting giant cell arteritis: a meta-analysis. Eur Radiol 32:3541–3552

    PubMed  Google Scholar 

  48. Duftner C, Dejaco C, Sepriano A, Falzon L, Schmidt WA, Ramiro S (2018) Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: a systematic literature review and meta-analysis informing the EULAR recommendations. RMD Open 4:e000612

    PubMed  PubMed Central  Google Scholar 

  49. Dejaco C, Ramiro S, Duftner C et al (2018) EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis 77:636–643

    PubMed  Google Scholar 

  50. Gaier ED, Gilbert AL, Cestari DM, Miller JB (2018) Optical coherence tomographic angiography identifies peripapillary microvascular dilation and focal non-perfusion in giant cell arteritis. Br J Ophthalmol 102:1141–1146

    PubMed  Google Scholar 

  51. Chen JJ, AbouChehade JE, Iezzi R Jr, Leavitt JA, Kardon RH (2017) Optical coherence angiographic demonstration of retinal changes from chronic optic neuropathies. Neuroophthalmology 41:76–83

    PubMed  PubMed Central  Google Scholar 

  52. Tuckwell K, Collinson N, Dimonaco S et al (2017) Newly diagnosed vs. relapsing giant cell arteritis: baseline data from the GiACTA trial. Semin Arthritis Rheum 46:657–664

    PubMed  Google Scholar 

  53. Brekke LK, Diamantopoulos AP, Fevang BT, Aβmus J, Esperø E, Gjesdal CG (2017) Incidence of giant cell arteritis in Western Norway 1972–2012: a retrospective cohort study. Arthritis Res Ther 19:278

    CAS  PubMed  PubMed Central  Google Scholar 

  54. Petursdottir V, Johansson H, Nordborg E, Nordborg C (1999) The epidemiology of biopsy-positive giant cell arteritis: special reference to cyclic fluctuations. Rheumatology (Oxford) 38:1208–1212

    CAS  PubMed  Google Scholar 

  55. Ashton-Key MR, Gallagher PJ (1992) False-negative temporal artery biopsy. Am J Surg Pathol 16:634–635

    CAS  PubMed  Google Scholar 

  56. López JM, Nazabal ER, Muñiz AM, Rey del Corral P (2001) Detection of posterior ischemic optic neuropathy due to giant cell arteritis with magnetic resonance imaging. Eur Neurol 46:109–110

    PubMed  Google Scholar 

  57. Botella-Ortiz AJ, Pampliega-Pérez A, Martín-Estefanía C, Alfaro-Sáez A, Concepción L, Teruel A (2004) The value of orbit magnetic resonance imaging in the diagnosis of blindness caused by giant cell arteritis. Rev Neurol 38:1149–1152

    CAS  PubMed  Google Scholar 

  58. Mitchell JR, Krashin-Bichler I, Rosenblum M, Diamond EL, Dinkin MJ (2014) Giant cell arteritis presenting with bilateral orbital inflammatory disease and enhancing superficial temporal arteries. Pract Neurol 14:446–447

    PubMed  PubMed Central  Google Scholar 

  59. Attaseth T, Vanikieti K, Poonyathalang A, Preechawat P, Jindahra P, Wattanatranon D (2015) Anterior ischemic optic neuropathy due to biopsy-proven giant cell arteritis in Thai patients. Clin Ophthalmol 9:1071–1075

    PubMed  PubMed Central  Google Scholar 

  60. Chen JJ, Kardon RH, Daley TJ, Longmuir RA (2015) Enhancement of the optic nerve sheath and temporal arteries from giant cell arteritis. Can J Ophthalmol 50:e96-97

    PubMed  Google Scholar 

  61. Mears KA, Feinsilber D (2020) High-sensitivity C-reactive protein and magnetic resonance imaging in occult giant cell arteritis. Cureus 12:e9530

    PubMed  PubMed Central  Google Scholar 

  62. Serrano Alcalá E, Grivé Isern E, Díez Borras L, Salaya Díaz JT (2020) Orbital Magnetic Resonance Imaging to unmask giant cell arteritis. Radiologia (Engl Ed) 62:160–163

    PubMed  Google Scholar 

  63. Panneerselvam S, Raviskanthan S, Mortensen PW, Lee AG (2021) giant cell arteritis presenting as central retinal artery occlusion and optic perineuritis: the importance of contrast MRI of the orbit. J Neuroophthalmol. https://doi.org/10.1097/wno.0000000000001415

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

Morgan P. Burke, BS is acknowledged for help with retrieving articles.

Funding

This study has received funding from the American Heart Association Career Development Award 938082 (Jae W Song, MD, MS) and NIH/NEI K08EY029765 (Qi N Cui, MD, PhD).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jae W. Song.

Ethics declarations

Guarantor

The scientific guarantor of this publication is Jae W. Song, MD, MS.

Conflict of interest

The authors of this manuscript declare relationships with the following companies: Julien Savatovsky, MD reports fees to prepare and give lectures by Canon, Novartis; Boards: Bayer, Biogen; Travel expenses: Bayer. Stocks: Chipiron and Guerbet.

Statistics and biometry

Quy Cao, PhD, kindly provided statistical advice for this manuscript.

Informed consent

Written informed consent was not required for this study because it is a literature-based systematic review.

Ethical approval

Institutional Review Board approval was not required because it is a literature-based systematic review of the literature.

Study subjects or cohorts overlap

Not applicable.

Methodology

Systematic Review and Individual Participant Data Meta-Analysis of the Literature. The PRIMSA guidelines were followed. This study was registered with PROSPER (Clinical trial registration no. CRD42021254494).

Additional information

Publisher's note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary information

Below is the link to the electronic supplementary material.

Supplementary file1 (PDF 349 KB)

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Guggenberger, K.V., Pavlou, A., Cao, Q. et al. Orbital magnetic resonance imaging of giant cell arteritis with ocular manifestations: a systematic review and individual participant data meta-analysis. Eur Radiol 33, 7913–7922 (2023). https://doi.org/10.1007/s00330-023-09770-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00330-023-09770-2

Keywords

Navigation