Systemic lupus erythematosus (SLE) is a rheumatic connective tissue autoimmune disease. It is a chronic, multi-system disease, with relapsing and remitting phases. Damage to various organs occurs in SLE when circulating immune complexes incite local inflammatory responses. Although the precise cause remains unknown, well-documented genetic predispositions have been identified (e.g., HLA-DR2 & HLA-DR3). Up to one third of patients with SLE may have the lupus anticoagulant, an antiphospholipid antibody. About half of those patients have the Antiphospholipid Antibody Syndrome, a condition associated with vaso-occlusive disease and thrombotic disorders. The syndrome may be a primary disease or may be secondary to SLE.
- Cutaneous disease occurs in most patients, with perhaps the most distinctive manifestation being the malar or ‘butterfly’ rash over the nose and cheeks.
- Kidney failure and infections are the leading causes of death in patients with SLE.
- Other areas of involvement in SLE include the skin & mucous membranes, joints (arthritis), serosal surfaces, central nervous & hematological systems.
Systemic symptoms during exacerbations may include malaise, fever, arthralgias and loss of appetite. The most common ocular symptom is burning or itching with keratoconjunctivitis sicca. Occasionally SLE causes severe loss of vision due to posterior segment complications.
- Anterior segment complications may include (a) periocular skin changes, (b) keratoconjunctivitis sicca, (c) scleritis and (d) a spectrum of corneal damage ranging from marginal thinning to ulceration and neovascularization.
- Retinal vascular lesions: Vascular changes in SLE may result from microangiopathy, possibly due to immune complexes interacting with the vessel walls, or may relate to systemic hypertension. The vasculitis, particularly with antiphospholipid antibodies, may lead to confluent areas of ischemia. Cotton wool spots and retinal hemorrhages are the most common signs. Progression to proliferative retinopathy is rare. Damage to choroidal vessels often results in multifocal serous retinal and retinal pigment epithelium detachments.
- Neurological effects: Vision may be affected by retrobulbar optic neuritis, anterior ischemic optic neuropathy, cranial nerve palsies and cerebral disease.
Rare (less than 1 per 10,000 per year), although more common in some populations. Up to 90 percent of patients are female.
Ocular complications of SLE tend to occur in patients with active systemic disease.
Hypertensive Retinopathy, Central Retinal Vein Occlusion, Behcet’s Disease, Sarcoidosis, Syphilis, Lyme Disease, Cytomegalovirus Retinitis, HIV retinopathy.
Branch Retinal Artery Occlusion, Retinal Detachment, Toxic Retinopathy – Chloroquine, Posterior Scleritis.
Antiphospholipid Antibody Syndrome may evolve rapidly, in a matter of days, requiring urgent treatment
Over 95 percent of patients test positive for antinuclear antibodies. Various additional antibody subtypes correlate with disease manifestations, e.g. Antiphospholipid antibodies, mentioned above.
Fluorescein angiography documents posterior segment inflammation & response to treatment, and informs decision-making when laser photocoagulation is being considered.
Being a rheumatic disease, the mainstay of medical therapy in SLE is systemic. Initial treatments include oral corticosteroids and non-steroidal anti-inflammatory medications. Disease control may require additional immunosuppressive agents (e.g., cyclophosphamide, azathioprine & hydroxychloroquine). Thrombotic complications are treated with long-term anticoagulant therapy.
Possible adverse ocular effects of systemic medications include cataract and glaucoma (corticosteroids) and macular toxicity (hydroxychloroquine), requiring at least annual review.
- Keratoconjunctivitis sicca is treated initially with tear supplements; additional treatments include punctual occlusion and pulse dose topical corticosteroids or cyclosporine.
- Laser photocoagulation may be appropriate in cases of retinal neovascularization.
- Vitreous hemorrhage and retinal detachment are treated with vitrectomy and scleral buckling.
Systemic lupus erythematosus with phopholipid antibodies leading to confluent areas of axoplasmic stasis with areas of increased nerve fibre swelling (bright white zones), where there is meeting point between antegrade and retrograde axoplasmic flow.
Fluorescein angiogram of the same patient in figure 1. Complete obliteration of the capillary bed and paucity of flow in the smaller arterioles, which appear like ghost vessels next to vessels filled with fluorescein.