DOI: 10.12890/2024_004345
Sudden visual loss due to arteritic anterior ischaemic optic neuropathy: A rare manifestation of eosinophilic granulomatosis with polyangiitis
Authors
antonio.faraone@uslcentro.toscana.it
Keywords
Anterior ischaemic optic neuropathy, eosinophilia, eosinophilic granulomatosis with polyangiitis, vasculitis, visual lossAbstract
Background: eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment.
Case report: we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x109 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture.
Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient’s visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment.
Conclusions: A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications.
References
- Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 2023;19:378-393.
- Akella SS, Schlachter DM, Black EH, Barmettler A. Ophthalmic eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): A systematic review of the literature. Ophthalmic Plast Reconstr Surg 2019;35:7-16.
- Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): State of the art. Allergy 2013;68:261-273.
- Hayreh SS. Ischaemic optic neuropathy. Prog Retin Eye Res 2009;28:34-62.
- Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis 2022;81:309-314.
- Ponte C, Grayson PC, Robson JC, Suppiah R, Gribbons KB, Judge A, et al. 2022 American College of Rheumatology/EULAR classification criteria for giant cell arteritis. Ann Rheum Dis 2022;81:1647-1653. Erratum in: Ann Rheum Dis 2023;82:e52.
- Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Rheumatol 2021;73:1349-1365.
- Nishimura T, Hosai M, Yamasaki R, Oiwa H. Temporal arteritis as an initial manifestation of eosinophilic granulomatosis with polyangiitis: A case report and a literature review. Mod Rheumatol Case Rep 2021;5:337-341.
- Hayreh SS. Management of ischaemic optic neuropathies. Indian J Ophthalmol 2011;59:123-136.
- Thurtell MJ, Kardon RH. Recovery of vision from no light perception in giant cell arteritis. Arch Ophthalmol 2012;1301080-1082.
