32-year-old female with systemic lupus erythematosus. Bilateral multiple cotton wool spots and retinal hemorrhage with vascular sheathing is noted (a, b), with vessel wall staining on fluorescein angiography (c, d)
Lupus Choroidopathy
Lupus choroidopathy can occur either independently or with lupus retinopathy, including single or multiple areas of serous or exudative retinal detachment, retinal pigment epithelium detachment or retinal pigment epitheliopathy (Nguyen et al. 2000). Subretinal hypopigmented patches on angiography which are areas of choroidal ischemia may also be present (Silpa-archa et al. 2016). Secondary angle closure glaucoma has also been reported from choroidal effusion leading to anterior shifting of the lens-iris diaphragm (Ahn and Choi 2016). Lupus choroidopathy resolves with appropriate immunosuppressive treatment and vision usually recovers (Nguyen et al. 2000).
Management
Systemic Management
Systemic treatment often requires a collaborative team approach. Nonsteroidal anti-inflammatory drugs, antimalarials (hydroxychloroquine), systemic corticosteroids, immunosuppressive therapy, and biologics may be effective. Systemic corticosteroids are used in the acute stage, and nonsteroidal anti-inflammatory drugs and hydroxychloroquine are used in mild patients. A variety of immunosuppressive agents have demonstrated efficacy in ocular SLE including methotrexate, mycophenolate mofetil, azathioprine, and cyclophosphamide (Palejwala et al. 2012; Silpa-archa et al. 2016). Biologics targeting cytokines, B and T lymphocytes, and B-cell-activating factors are also considered in patients refractory to other treatment. Belimumab is the first biologic agent approved by the Federal Drug Administration in 2011 for SLE, which inactivates B-cell-activating factor (Dooley et al. 2013; Navarra et al. 2011; Touma et al. 2013). Others include rituximab, epratuzumab, sifalimumab, and ocrelizumab.