Purtscher-like Retinopathy in SLE – October, 2024
History:
A 24-year-old female was referred for cotton wool spots OU. She complained of mild blurry vision with flashing lights OU. She was recently diagnosed with systemic lupus erythematosus (SLE) 2 weeks prior had been started on Plaquenil and 60mg prednisone.
Exam:
VA (Visual acuity) was 20/20 in both eyes (OU). Intraocular pressure was normal. Anterior exam was normal. Dilated fundus exam revealed venous tortuosity, Purtscher-like spots in the posterior pole with mild disc edema OU (Fig 1 + 2). Optical Coherence Tomography OCT demonstrated disc edema OU and peripapillary subretinal fluid OD (Fig 3). Fluorescein angiogram showed multifocal hyperfluorescence with peripapillary late pooling OU (Fig 4 + 5).
Clinical Course:
She was sent to the emergency room for work up of bilateral disc swelling. She experienced status epilepticus requiring ICU admission. She was diagnosed with CNS lupus, hypertension, nephrotic syndrome, and hemolytic anemia. Her subretinal fluid resolved with continued treatment of lupus and management of blood pressure. She remained was discharged with a plan to start immunomodulating therapy.
Differential Diagnosis:
• Hypertensive retinopathy
• Lupus retinopathy
• Purtscher-like Retinopathy in SLE
Discussion:
Purtscher-like Retinopathy in SLE
SLE is an autoimmune condition characterized by overactivation of the immune system with a predominance in females. Onset is typically at 20-30 years of age. It is a systemic disease with 40% having renal involvement and about 33-50% having ocular involvement. Ocular involvement can be in the form of keratoconjunctivitis sicca, scleritis, extraocular muscle disorders, and retinopathy.
Lupus retinopathy is more likely in severe disease and can present with cotton wool spots, retinal hemorrhages, and vascular abnormalities.
Management involves SLE control by a rheumatologist and management of other systemic manifestations. Due to the potentially life-threatening association of CNS involvement with Purtscher-like retinopathy in SLE, it is therefore imperative to send patients for a systemic work up as soon as possible.
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References:
- Silpa-archa, S., Lee, J. J., & Foster, C. S. (2015). Ocular manifestations in systemic lupus erythematosus. British Journal of Ophthalmology, 100(1), 135–141.
- Ushiyama, O. (2000). Retinal disease in patients with systemic lupus erythematosus. Annals of the Rheumatic Diseases, 59(9), 705–708.
- Wu C, Dai R, Dong F, Wang Q. Purtscher-like retinopathy in systemic lupus erythematosus. Am J Ophthalmol. 2014 Dec;158(6):1335-1341.e1. doi: 10.1016/j.ajo.2014.09.001. Epub 2014 Sep 6. PMID: 25205559.