Severe vaso-occlusive lupus retinopathy

Rationale: Purtscher-like retinopathy is an extremely rare, vision-threatening microvascular complication of systemic lupus erythematosus (SLE). To date, there is no standardized, unified treatment guideline for this condition worldwide, and conventional interventions including systemic glucocorticoids, immunosuppressants, and anti- vascular endothelial growth factor therapy often yield unsatisfactory visual outcomes. Additionally, the early ocular manifestations of SLE are easily overlooked due to atypical systemic symptoms, leading to delayed diagnosis and irreversible visual impairment. Patient concerns: A 24-year-old female developed blurred vision after coronavirus disease 2019 infection, which was initially neglected. She subsequently presented with persistent fever, lymphadenopathy, recurrent seizures and altered consciousness, and was diagnosed with SLE complicated by neuropsychiatric lupus. Her visual loss progressively aggravated during systemic treatment. Diagnoses: Systemic workup confirmed active SLE with neuropsychiatric involvement. Serial ophthalmic examinations revealed bilateral Purtscher-like retinopathy characterized by venous tortuosity, extensive cotton-wool spots, retinal hemorrhages, macular edema, diffuse capillary non-perfusion, and foveal arteriovenous anastomosis, with secondary right eye vitreous hemorrhage. Interventions: On the basis of standard SLE treatment with glucocorticoids and immunosuppressants, comprehensive interventions were implemented, including blood pressure optimization, anticoagulant discontinuation to mitigate bleeding risk, fractional panretinal photocoagulation for retinal ischemia, and adjunctive modified Ditan Decoction based on Traditional Chinese Medicine syndrome differentiation. Outcomes: During follow-up, blood pressure was gradually stabilized. Right eye vitreous hemorrhage resolved completely without recurrent bleeding. Retinal ischemia improved after photocoagulation, with partial regression of foveal arteriovenous anastomosis. Visual acuity improved modestly and remained stable at the final assessment. Lessons: Ocular microvascular lesions may precede systemic disease flares in SLE, underscoring the necessity of routine ophthalmologic screening for newly diagnosed patients, particularly those with neuropsychiatric involvement. Adjunctive Traditional Chinese Medicine therapy may offer incremental benefit for refractory Purtscher-like retinopathy, representing a complementary strategy that warrants further investigation.

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Publication Details

Journal
Medicine
Published
2026-09-04
DOI
https://doi.org/10.1097/md.0000000000050535
Primary Topic
Retinal and Optic Conditions
Type
article
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0.00
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article

Severe vaso-occlusive lupus retinopathy

Xiaoyang Xie, Huili Li, Kaiyao Chi, Tong Guo et al.
Medicine
Retinal and Optic Conditions
article

Severe vaso-occlusive lupus retinopathy

Xiaoyang Xie, Huili Li, Kaiyao Chi, Tong Guo, Daoxi Lei
article en

Abstract

Rationale: Purtscher-like retinopathy is an extremely rare, vision-threatening microvascular complication of systemic lupus erythematosus (SLE). To date, there is no standardized, unified treatment guideline for this condition worldwide, and conventional interventions including systemic glucocorticoids, immunosuppressants, and anti- vascular endothelial growth factor therapy often yield unsatisfactory visual outcomes. Additionally, the early ocular manifestations of SLE are easily overlooked due to atypical systemic symptoms, leading to delayed diagnosis and irreversible visual impairment. Patient concerns: A 24-year-old female developed blurred vision after coronavirus disease 2019 infection, which was initially neglected. She subsequently presented with persistent fever, lymphadenopathy, recurrent seizures and altered consciousness, and was diagnosed with SLE complicated by neuropsychiatric lupus. Her visual loss progressively aggravated during systemic treatment. Diagnoses: Systemic workup confirmed active SLE with neuropsychiatric involvement. Serial ophthalmic examinations revealed bilateral Purtscher-like retinopathy characterized by venous tortuosity, extensive cotton-wool spots, retinal hemorrhages, macular edema, diffuse capillary non-perfusion, and foveal arteriovenous anastomosis, with secondary right eye vitreous hemorrhage. Interventions: On the basis of standard SLE treatment with glucocorticoids and immunosuppressants, comprehensive interventions were implemented, including blood pressure optimization, anticoagulant discontinuation to mitigate bleeding risk, fractional panretinal photocoagulation for retinal ischemia, and adjunctive modified Ditan Decoction based on Traditional Chinese Medicine syndrome differentiation. Outcomes: During follow-up, blood pressure was gradually stabilized. Right eye vitreous hemorrhage resolved completely without recurrent bleeding. Retinal ischemia improved after photocoagulation, with partial regression of foveal arteriovenous anastomosis. Visual acuity improved modestly and remained stable at the final assessment. Lessons: Ocular microvascular lesions may precede systemic disease flares in SLE, underscoring the necessity of routine ophthalmologic screening for newly diagnosed patients, particularly those with neuropsychiatric involvement. Adjunctive Traditional Chinese Medicine therapy may offer incremental benefit for refractory Purtscher-like retinopathy, representing a complementary strategy that warrants further investigation.

MedicineVol. 105(36)
The Affiliated Yongchuan Hospital of Chongqing Medical University (CN), First People's Hospital of Chongqing (CN), Chengdu University of Traditional Chinese Medicine (CN), Chongqing Medical University (CN)
Good health and well-being
Openalex Percentile: Top 8%
Retinal and Optic Conditions
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