Autoimmune Retinopathy in Systemic Lupus Erythematosus: Histopathologic Features

Xiaoguang Cao1, Rachel J Bishop2, Farzin Forooghian2, Youngeun Cho1, Robert N Fariss3, Chi-Chao Chan*, 1
1 Immunopathology Section, Laboratory of Immunology,Bethesda, MD 20892, USA
2 Clinical Branch, National Institutes of Health, Bethesda, MD 20892, USA
3 Imaging Core, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA

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© Cao et al.; Licensee Bentham Open.

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* Address correspondence to this author at the Immunopathology Section, Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA; E-mail:


The ocular pathology of autoimmune retinopathy is demonstrated in a 62-year-old female patient with systemic lupus erythematosus (SLE) who presented with typical clinical autoimmune retinopathy. Macroscopically, there were multiple depigmented lesions in the peripheral retina and choroid and scattered pigmentary bone-spickling at the equator and periphery. Microscopically, there were generalized loss of photoreceptors and thinning of the outer plexiform layer. Many peripheral retinal vessels were sclerotic and occluded, some surrounded by pigment granules and RPE cells. Cobblestone degeneration was prominent in the periphery. Macrophages were seen in the retina, particularly in areas of photoreceptor degeneration. Rare, scattered T- lymphocytes were present in the retina and choroid, while B-cells were notably absent. The optic nerve showed loss of axons and thickened septae. Serum autoantibodies against normal retinal nuclei were detected. These pathological changes represent both known SLE-associated ocular complications as well as possible features of autoimmune retinopathy secondary to SLE.