J Rheum Dis.  2022 Jan;29(1):52-55. 10.4078/jrd.2022.29.1.52.

Ischemic Vaso-occlusive Retinopathy as Initial Presentation in Pediatric Systemic Lupus Erythematosus: A Case Report

Affiliations
  • 1Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea

Abstract

Ischemic vaso-occlusive retinopathy as an initial manifestation is rare in pediatric systemic lupus erythematosus (pSLE). A 13-year-old girl presented with two months’ history of papules and crusts with fatigue, weight loss, and abrupt hair loss. Pancytopenia and findings compatible with SLE, including positive direct Coombs’ test, antinuclear antibody (Ab), anti-double stranded DNA Ab, anti-Smith Ab, anti-ribonucleoprotein Ab, lupus anticoagulant, anti-β2 glycoprotein Immunoglobulin G, and anti-cardiolipin Ab, were detected. Bi-nasal hemianopsia was detected. Initial visual acuity was hand motion in the right eye and 15/20 in the left. Fundoscopy showed massive exudation around the optic disc with macular edema, vascular sheathing with perivascular hemorrhage in the whole retina, and ghost vessels in the peripheral retina. Intravitreal triamcinolone injection and dexamethasone implant injection were administered. Visual symptoms improved but did not recover. Methylprednisolone therapy and photocoagulation improved visual acuity and fever. Early intervention for retinopathy in pSLE can help prevent vision-loss.

Keyword

Lupus erythematosus; systemic; Pediatrics; Retinal diseases

Figure

  • Figure 1 Macular optical coherence tomographs. Massive exudation at both eyes with macular edema was noted. Right eye (A), left eye (B).

  • Figure 2 Fundoscopic findings showed vascular sheathing with perivascular hemorrhage at whole retina and ghost vessels at periphery retina at both eyes.


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