Contributor: Gordon K. Klintworth
Systemic lupus erythematosus (lupus erythematosus, SLE) is a chronic systemic autoimmune disease involving cellular and humoral immunity. B-Cell hyperactivity is present and numerous non-organ specific autoantibodies are present, including antinuclear antibodies. Antibodies to double-stranded DNA, ribosomal P protein, and Sm protein are very specific for systemic lupus erythematosus but not particularly sensitive. The manifestations include fever and affect the skin (erythematous rash), joints (polyarthralgia and arthritis) polyserositis (especially pleurisy), blood (anemia, thrombocytopenia), kidney (renal lesions in the glomeruli, renal tubules and blood vessels), nervous system (neurologic abnormalities) and heart (pericarditis, Libman-Sacks endocarditis). A retinopathy in the posterior pole with microinfarctions of the nerve fiber layer secondary to occlusovascular disease with is common (~7% of patients. Microangiopathy (~83% of patients), transient papilledema, ischemic optic neuropathy [optic neuropathy - ischemic], and  central retinal vein occlusion. Immunoglobulin deposits in vascular basement membranes, including the renal glomerulus. This is followed by the binding of complement and the complex may lead to a lysis of vascular endothelial cell membranes and a release of enzymes from neutrophils. Various thrombogenic antibodies (lupus anticoagulant, anticardiolipin antibodies, and other antiphospholipid antibodies) contribute vascular occlusions. The affected retina may contain numerous zones with discrete, thickened precapillary retinal arterioles surrounding cotton-wool spots. Perivasculitis of the peripheral retinal vessels, hemorrhage, vascular necrosis, papilledema, central retinal artery occlusion, choroidal occlusovascular disease and a focal leakage of retinal pigment epithelium with secondary localized retinal detachment may occur.