Autoimmune Retinopathy in Systemic Lupus Erythematosus: Histopathologic Features

Abstract

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.

Sudden acquired retinal degeneration syndrome (SARDS) – a review and proposed strategies toward a better understanding of pathogenesis, early diagnosis, and therapy

“…Similar to SARDS, AIR is not associated with any clinical signs of intraocular inflammation . Except for a few macrophages in the regions of retinal cell loss, no significant inflammation is seen histologically within AIR‐affected retinas . However, T and B lymphocytes probably contribute to the disease process as suggested by documented activation of the complement system .…”

Autoimmune retinopathy associated with systemic lupus erythematosus: A diagnostic dilemma

“…[12] AIR is characterized by cone and rod dysfunction associated with photoaversion, subacute progressive loss of vision, nyctalopia, abnormalities of color perception, central or paracentral scotoma, and decreased photopic and scotopic responses on ERG. [2] npAIR shares the same features as CAR/MAR but occurs in the absence of malignant tumor.…”

“…Three subtypes have been identified: cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and nonparaneoplastic AIR (npAIR). [12] AIR is characterized by acute or subacute progressive visual deterioration, visual field loss, abnormal electroretinography (ERG), the presence of circulating antiretinal autoantibodies (ARAs), and often a normal appearing retina. [2]…”

“…Previously reported posterior segment manifestations include retinal vasculitis or vascular occlusion, choroidopathy, optic neuritis, and toxic retinopathy related to the use of hydroxychloroquine in SLE treatment. [13] Reports of SLE-associated AIR are rare and may be incorrectly ascribed to hydroxychloroquine toxicity. [1]…”

“…[13] Reports of SLE-associated AIR are rare and may be incorrectly ascribed to hydroxychloroquine toxicity. [1]…”