Purtscher-Like Retinopathy as an Atypical Initial Manifestation of Systemic Lupus Erythematosus with Vaso-Occlusive Vasculitis: Case Report

Authors

DOI:

https://doi.org/10.48560/rspo.44543

Keywords:

Antiphospholipid Syndrome, Lupus Erythematosus, Systemic, Retinal Diseases, Retinal Vasculitis

Abstract

Purtscher-like retinopathy is a rare retinal vasculopathy associated with non-traumatic systemic conditions, including autoimmune diseases. It may present as an early manifestation of systemic lupus erythematosus (SLE), particularly in the presence of antiphospholipid antibodies. We report a 29-year-old woman who presented with bilateral visual loss. Fundoscopy showed cotton wool spots, Purtscher flecken, marked retinal edema, and intraretinal hemorrhages. Optical coherence tomography demonstrated inner retinal hyperreflectivity and cystoid macular edema, and fluorescein angiography revealed macular and peripheral non-perfusion. Laboratory testing showed positive antinuclear antibodies (1:2560), anti-dsDNA, anti-ribosomal P antibodies, low complement levels and antiphospholipid antibodies, supporting the diagnosis of SLE with probable secondary antiphospholipid syndrome. Treatment with systemic corticosteroids, rituximab, and intravitreal dexamethasone resulted in limited visual recovery. This case highlights Purtscher-like retinopathy as a severe initial manifestation of SLE and underscores the importance of early recognition to prevent significant complications.

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References

Miguel AI, Henriques F, Azevedo LF, Loureiro AJ, Maberley DA. Systematic review of Purtscher's and Purtscher-like retinopathies. Eye. 2013;27:1-13. doi:10.1038/eye.2012.222

Agrawal A, McKibbin M. Purtscher's and Purtscher-like retinopathies: a review. Surv Ophthalmol. 2006;51:129-36. doi:10.1016/j.survophthal.2005.12.003

Wu C, Dai R, Dong F, Wang Q. Purtscher-like retinopathy in systemic lupus erythematosus. Am J Ophthalmol. 2014;158:1335-41.e1. doi:10.1016/j.ajo.2014.09.001

Kunavisarut P, Pathanapitoon K, Rothova A. Putscher-like retinopathy associated with systemic lupus erythematosus. Ocul Immunol Inflamm. 2016;24:60-8. doi:10.3109/09273948.2014.932816

Jabs DA, Fine SL, Hochberg MC, Newman SA, Heiner GG, Stevens MB. Severe retinal vaso-occlusive disease in systemic lupus erythematosus. Arch Ophthalmol. 1986;104:558-63. doi:10.1001/archoph.1986.01050160074027

D'Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet. 2007;369:587-96. doi:10.1016/S0140-6736(07)60279-7

Petri M. Epidemiology of systemic lupus erythematosus. Best Pract Res Clin Rheumatol. 2002;16:847-58. doi:10.1053/berh.2002.0259

Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: a review of the literature. Autoimmune Dis. 2012;2012:290898. doi:10.1155/2012/290898

Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm. 2004;12:87-99. doi:10.1080/09273940409895308

Alahmadi RM, Hashim RT, Almogairin SM, Abu AM. Purtscher-like retinopathy as a first presentation of systemic lupus erythematosus. Ann Saudi Med. 2016;36:85-8. doi:10.5144/0256-4947.2016.85

Meng L, Yu Q, Zhao X, Chen L, Wang Y, Zhang W, et al. Purtscher-like retinopathy in systemic lupus erythematosus: clinical features, risk factors and prognosis. QJM. 2023;116:923-32. doi:10.1093/qjmed/hcad204

Mejia-Turizo JC, Duran-Merino CE, Gomez-Rodriguez PA, Vanegas-Ramirez CM. Purtscher-like retinopathy with bilateral vasculitis and vascular occlusion as the first manifestation of systemic lupus erythematosus: a case report. Rev Colomb Reumatol. 2023;30:1-6. doi:10.1016/j.rcreue.2021.05.003

Garcia-Soler E, Martinez-Rubio C, De Smet MD, Udaondo P, Salom-Alonso D. Systemic lupus erythematosus Purtscherlike retinopathy: optical coherence tomography angiography assessment implications. Eur J Ophthalmol. 2023;34:N75-9. doi:10.1177/11206721231171705

Dammacco R. Systemic lupus erythematosus and ocular involvement: an overview. Clin Exp Med. 2018;18:135-49. doi:10.1007/s10238-017-0479-9

Lee DH, Hwang CK, Cukras CA, Wiley HE, Malik FF, Chew EY, et al. Two cases of severe Purtscher-like retinopathy demonstrating recurrence and progression to neovascularization and vitreous hemorrhage. Am J Ophthalmol Case Rep. 2020;18:100664. doi:10.1016/j.ajoc.2020.100664

Fanouriakis A, Kostopoulou M, Andersen J, Aringer M, Arnaud L, Bae SC, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis. 2024;83:15-29. doi:10.1136/ard-2023-224762

Tan S, Mai S, Yang J. Risk assessment of infertility due to cyclophosphamide use for premenopausal women with systemic lupus erythematosus: a meta-analysis. Int J Clin Exp Med. 2016;9:19011-8.

Pendergraft WF 3rd, McGrath MM, Murphy AP, Murphy P, Laliberte KA, Greene MF, et al. Fetal outcomes after rituximab exposure in women with autoimmune vasculitis. Ann Rheum Dis. 2013;72:2051-3. doi:10.1136/annrheumdis-2013-203833

Ejaz K, Abid D, Juneau P, Chu J, Hasni S. Use of gonadotropin-releasing hormone agonists for ovarian preservation in patients receiving cyclophosphamide for systemic lupus erythematosus: a meta-analysis. Lupus. 2022;31:1706-13. doi:10.1177/09612033221128740

Murray JJ, Lyons CJ, Lee-Wing MW. Re: Marmor et al.: American Academy of Ophthalmology Statement: Recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 Revision). Ophthalmology. 2017;124:e28-9. doi:10.1016/j.ophtha.2016.11.002

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Published

2026-06-13

How to Cite

Sampaio, M., Sousa, K., Costa, J., Capela, C., & Palha, V. (2026). Purtscher-Like Retinopathy as an Atypical Initial Manifestation of Systemic Lupus Erythematosus with Vaso-Occlusive Vasculitis: Case Report. Revista Sociedade Portuguesa De Oftalmologia, 50(2), 172–177. https://doi.org/10.48560/rspo.44543

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Case Reports