Purtscher-Like Retinopathy as an Atypical Initial Manifestation of Systemic Lupus Erythematosus with Vaso-Occlusive Vasculitis: Case Report
DOI:
https://doi.org/10.48560/rspo.44543Keywords:
Antiphospholipid Syndrome, Lupus Erythematosus, Systemic, Retinal Diseases, Retinal VasculitisAbstract
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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