Journal of Ophthalmic and Vision Research
ISSN: 2008-322X
The latest research in clinical ophthalmology and vision science
Orbital Presentation of Eosinophilic Granulomatosis with Polyangiitis: An Interventional Case Report and Literature Review
Published date: Aug 27 2025
Journal Title: Journal of Ophthalmic and Vision Research
Issue title: Volume 20 - 2025
Pages: 1 - 6
Authors:
Abstract:
Purpose: To report a case of eosinophilic granulomatosis with polyangiitis (EGPA) initially presenting as orbital involvement, describe its successful management, and provide a comprehensive literature review.
Case Report: A 33-year-old female patient presented with swelling, redness, tenderness, and a mass under the left upper eyelid for one month. Upper lid eversion showed a multilobulated lesion in the subconjunctival area of the same region. The patient’s medical history included asthma and atrial septal defect surgery. Orbital MRI revealed a soft tissue mass infiltrating the superior and lateral aspects of the conal and extraconal regions in the anterior orbit, with extension toward the preseptal area. The lesion underwent incisional biopsy, and histopathological findings were consistent with the diagnosis of EGPA. The patient’s blood tests revealed eosinophilia and a negative antineutrophil cytoplasmic antibody. After excluding other similar pathologies such as granulomatosis with polyangiitis, we observed a dramatic regression in her orbital lesion following systemic steroid therapy.
Conclusion: The diagnosis of EGPA, a rare clinical presentation, is crucial for ophthalmologists because it provides early recognition of the systemic disease and can help slow its progression by initiating appropriate treatment in a timely manner.
Keywords: Antineutrophil Cytoplasmic Antibody, Eosinophilia, Eosinophilic Granulomatosis with Polyangiitis, Orbit, Systemic Steroid
References:
1. Churg J. Allergic graulomatosis and granulomatous vascular syndromes. Ann Allergy 1963;21:619–628.
2. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1–11.
3. Keogh KA, Specks U. Churg-Strauss syndrome: Clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003;115:284–290.
4. 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.
5. Sale S, Patterson R. Recurrent Churg-Strauss vasculitis. With exophthalmos, hearing loss, nasal obstruction, amyloid deposits, hyperimmunoglobulinemia E, and circulating immune complexes. Arch Intern Med 1981;141:1363–1365.
6. Chakraborty RK, Aeddula NR. Eosinophilic granulomatosis with polyangiitis (Churg- Strauss Syndrome). StatPearls Publishing; 2024. http://www.ncbi.nlm.nih.gov/books/NBK537099/
7. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277– 301.
8. Zwerina J, Bach C, Martorana D, Jatzwauk M, Hegasy G, Moosig F, et al. Eotaxin-3 in Churg-Strauss syndrome: A clinical and immunogenetic study. Rheumatology (Oxford) 2011;50:1823–1827.
9. Radice A, Bianchi L, Sinico RA. Anti-neutrophil cytoplasmic autoantibodies: Methodological aspects and clinical significance in systemic vasculitis. Autoimmun Rev 2013;12:487–495.
10. Rothschild PR, Pagnoux C, Seror R, Brézin AP, Delair E, Guillevin L. Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: A retrospective study of 1286 patients and review of the literature. Semin Arthritis Rheum 2013;42:507–514.
11. Fijolek J, Radzikowska E. Eosinophilic granulomatosis with polyangiitis - Advances in pathogenesis, diagnosis, and treatment. Front Med (Lausanne) 2023;10:1145257.
12. Quan MV, Frankel SK, Maleki-Fischbach M, Tan LD. A rare case report of polyangiitis overlap syndrome: Granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. BMC Pulm Med 2018;18:181.
13. Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome. Autoimmun Rev 2015;14:341–348.
14. Takanashi T, Uchida S, Arita M, Okada M, Kashii S. Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: Report of two cases and review of the literature. Ophthalmology 2001;108:1129–1133.
15. Vitali C, Genovesi-Ebert F, Romani A, Jeracitano G, Nardi M. Ophthalmological and neuro-ophthalmological involvement in Churg-Strauss syndrome: A case report. Graefes Arch Clin Exp Ophthalmol 1996;234:404–408.
16. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094–1100.
17. White J, Dubey S. Eosinophilic granulomatosis with polyangiitis: A review. Autoimmun Rev 2023;22:103219.
18. Albreiki D, Al Belushi F, Patel V, Farmer J. When a temporal artery biopsy reveals a diagnosis other than temporal arteritis: Eosinophilic granulomatosis with polyangiitis. Can J Ophthalmol 2016;51:e108–109.
19. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: Assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 2011;90:19–27.
20. Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg- Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med 2015;26:545–553.
21. Ford JA, Aleatany Y, Gewurz-Singer O. Therapeutic advances in eosinophilic granulomatosis with polyangiitis. Curr Opin Rheumatol 2022;34:158–164.