Table 3:

Therapeutic options in retinopathy and retinal vasculitis in systemic lupus erythematosus*

Corticosteroids1,2Considered as initial therapy for most patients
Periocular corticosteroid injections can be used in mild to moderate unilateral disease
Systemic corticosteroids are reserved for moderate to severe ocular involvement: oral prednisone 1–2 mg/kg per day, with or without initial pulse therapy of intravenous methylprednisolone (1 g per day for 3–6 days)
Many patients will show clinical improvement only 3–4 weeks after starting treatment, regardless of treatment schedule used
Other immunosuppressive medications1,2,7Cyclophosphamide, mycophenolate mofetil, azathioprine, chlorambucil, cyclosporine, tacrolimus and methotrexate have been used as steroid-sparing agents or for patients with substantial retinal disease who do not respond to systemic steroid therapy alone
In severe vaso-occlusive disease, which may be a manifestation of antiphospholipid syndrome, immunosuppressive treatment is not useful
Plasmapheresis1,7May be tried in severe retinal vasculitis, usually combined with immunosuppressive therapy (cyclophosphamide or methotrexate)
Aspirin and/or anticoagulation therapy1Used with variable degrees of success in patients with severe vaso-occlusive retinopathy associated with antiphospholipid syndrome
Rituximab (anti-CD20 monoclonal antibody)8Single case report of successful use for retinal vasculitis in a patient with systemic lupus erythematosus with improvement in vision
Panretinal laser photocoagulation and vitreoretinal surgical procedures1,2These procedures are used to treat complications associated with retinal vasculitis (neovascularization, vitreous hemorrhage and neovascular glaucoma) to limit further loss of vision
Immunosuppressive therapy should be used to achieve control of intraocular inflammation before start of laser treatment
  • * Optimal treatment is not well defined owing to a lack of well-designed clinical trials.