Systemic Lupus Erythematosus Presenting as Acute Posterior Multifocal Placoid Pigment Epitheliopathy - Case Report and Review of the Ocular Manifestations of Systemic Lupus Erythematosus

Abstract

The ocular manifestations of systemic lupus erythematosus (SLE) are numerous and may be due to complement-activating immune complex deposition causing inflammation or thrombosis, secondary effects of SLE such as hypertension, related diseases such as Sjogren’s and Antiphospholipid antibody syndrome, or a combination of these. Left untreated, these manifestations can result in serious morbidity and rarely, mortality. Here we present the first reported case of systemic lupus erythematosus masquerading as Acute Posterior Multifocal Placoid Pigment Epitheliopathy, a rare chorioretinal disorder. An overview of the numerous ocular manifestations of this disease are presented, as well as recent highlights into the pathophysiology of SLE. Case Report Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare disease affecting males and females equally in their second to third decade of life. It is thought to be due to an immune driven vascular alteration of the choriocapillaris with a secondary pigment epithelial reaction. More than half are associated with a viral etiology, and up to one-third will have a viral prodrome. Systemic autoimmune diseases such as erythema nodosum, granulomatosis with polyangiitis (GPA), polyarteritis nodosa (PAN), as well as other inflammatory diseases such as cerebral vasculitis, microvascular nephropathy, scleritis and episcleritis are associated with APMPPE [1,2]. A 35 year-old Caucasian female with a known history of systemic lupus erythematosus presented with a two week history of decreased vision and headaches. She had been previously well with no symptoms of infection. Her visual acuity at presentation was 20/500 right eye and 20/30 left eye, with intraocular pressures of 12 mmHg in both eyes and no afferent pupillary defect. Diffuse anterior scleritis of the right eye was noted following instillation of phenylephrine 10%. Funduscopy revealed bilateral confluent, deep, cream-colored chorioretinal lesions of varying sizes with indistinct borders located mostly between the