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
Highlights
There were atypical features including profuse vascular leakage and punctate hyperfluorescence in the temporal macula and around the optic nerve
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 Acute posterior multifocal placoid pigment epitheliopathy (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
Summary
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that preferentially affects women in their childbearing years. While the diagnostic criteria for SLE by the American College of Rheumatology from 1997 are still being used, the Systemic Lupus International Collaborating Clinics (SLICC) group recently revised the criteria in 2012 to include seventeen clinical or laboratory criteria. Diagnosis requires at least 4 criteria, including at least one clinical criterion and one immunologic criterion, or a positive biopsy-proven lupus nephritis in the presence of antinuclear antibodies or anti-double-stranded DNA antibodies [4]. 1. Acute cutaneous lupus, including: lupus malar rash, bullous lupus, toxic epidermal necrolysis variant of SLE, maculopapular lupus rash, photosensitive lupus rash (in the absence of dermatomyositis), or subacute cutaneous lupus. Synovitis involving 2 or more joints, characterized by swelling or effusion or tenderness in 2 or more joints and at least 30 minutes of morning stiffness
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