Treatment options for SLE range from nonsteroidal anti-inflammatory drugs, corticosteroids, antimalarials, immunomodulatory, and biologic agents. Significant ocular involvement—orbital inflammation, scleritis, retinal vasculitis, choroiditis, and optic neuritis—warrants systemic therapy. The goal of treatment is to suppress immune activity, specifically decreasing the level of autoantibodies.
Corticosteroids are the mainstay and most effective short-term therapy for SLE [80
]. They inhibit both the innate and adaptive immune response by preventing proliferation and inducing apoptosis of T cells, B cells, and macrophages as well as reducing levels of cytokines and prostaglandins [81
]. Generally, by the time patients present with ocular manifestation, they have a high level of systemic inflammation. Previous papers have shown that a high correlation exists between CNS vasculitis and retinal vasculitis [49
] as well as nephritis and choroiditis [56
]. Nguyen et al. recently described a series of four of 28 patients with choroidopathy who died from lupus-related complications [83
]. Early and aggressive treatment is needed for this group to prevent increased morbidity and mortality. Thus, it is of extreme importance that patients presenting with severe ocular manifestations be treated with high-dose oral or even IV steroids early on in the disease course. Periocular steroid injections may have a role in unilateral/asymmetric disease; however, they should be used cautiously and avoided in patients with scleritis.
Steroid-sparing immunosuppressive agents are used in a large amount of patients secondary to treatment failure or harmful side effects of corticosteroids. Antimalarials such as chloroquine and more commonly hydroxychloroquine are often used. These medications are highly efficacious in curtailing future flares with fewer side effects compared to other immunomodulatory drugs such as alkylating agents. However, ocular effects of these drugs are well known. Irreversible vision loss secondary to a drug-induced maculopathy has been well documented in the literature. Factors associated with high risk of developing maculopathy include greater than 5–7 years of therapy, greater than a cumulative dose of 1000
g of hydroxychloroquine, impairment of liver or kidney function, obesity, age greater than 65, and preexisting retinopathy. The American Academy of Ophthalmology recommends a baseline-dilated eye exam on all patients starting hydroxychloroquine followed by annual exams starting at 5 years after initiating therapy. A Humphrey 10–2 automated visual field test along with multi-focal electroretinogram, spectral domain optical coherence tomography, or fundus autofluorescence should be performed at each of these visits. Discontinuation of the drug should be recommended at the earliest sign of toxicity [84
]. Unfortunately, cases of progression of retinopathy despite cessation of therapy have been reported [85
Methotrexate, azathioprine, mycophenolate mofetil, cyclosporine A, cyclophosphamide, and chlorambucil have all been employed with varying degrees of success.
In the past few years, newer drugs, categorized as biological agents, have emerged targeting specific molecules involved in B- and T-cell activation. One of the first to be utilized in SLE was rituximab, a chimeric murine/human anti-CD20 antibody. Multiple studies have shown clinical improvement in refractory patients [86
]. Rituximab has also shown efficacy in treating noninfectious forms of ocular inflammation including that secondary to SLE [88