What medications are used to manage a Systemic Lupus Erythematosus (SLE) flare-up in the emergency department (ED)?

From the Guidelines

For managing a lupus flare in the emergency department, the initial treatment should include high-dose glucocorticoids, such as pulses of intravenous methylprednisolone (usually 250–1000 mg per day, for 1–3 days), as recommended by the 2019 EULAR guidelines 1. This approach is based on the guidelines' suggestion that glucocorticoids can be used at doses and route of administration that depend on the type and severity of organ involvement, with a high level of agreement among experts (9.95/10) 1. The use of high-dose glucocorticoids is supported by the guidelines' statement that pulses of intravenous methylprednisolone provide an immediate therapeutic effect and enable the use of lower starting doses of oral glucocorticoids (9.85/10) 1. In addition to glucocorticoids, other medications such as hydroxychloroquine (HCQ) may be considered, as it is recommended for all patients with SLE, unless contraindicated, at a dose not exceeding 5 mg/kg/real body weight (9.65/10) 1. For organ-threatening disease, immunosuppressive agents such as cyclophosphamide or mycophenolate mofetil may be considered, as recommended by the guidelines (9.85/10 and 9.90/10, respectively) 1. Some key points to consider when managing a lupus flare in the emergency department include:

• Assessing disease activity and organ involvement through laboratory tests
• Providing supportive care, such as IV fluids for dehydration, antiemetics for nausea, and pain management
• Tailoring the treatment approach to the specific organs involved and severity of the flare
• Consulting with rheumatology for follow-up care The treatment of lupus flares should aim to reduce inflammation, prevent organ damage, and improve quality of life, while minimizing the risk of adverse effects from medications. The guidelines emphasize the importance of prompt initiation of immunomodulatory agents to expedite the tapering or discontinuation of glucocorticoids (9.90/10) 1. Overall, the management of lupus flares in the emergency department requires a comprehensive approach that takes into account the severity of the flare, the specific organs involved, and the patient's overall health status.

From the Research

Medications for Lupus Flare-up in the Emergency Department

• Corticosteroids are a mainstay of therapy for severe organ-threatening systemic lupus erythematosus, including lupus nephritis and central nervous system (CNS) lupus 2
• The current standard of care for patients with lupus nephritis is treatment with a combination of steroids plus either mycophenolate mofetil (MMF) or cyclophosphamide 3
• Intravenous (IV) pulses of methylprednisolone (MEP) are commonly used to treat severe manifestations of systemic lupus erythematosus (SLE), including lupus nephritis and CNS lupus 4

Treatment Protocols

• The Lupus-Cruces nephritis protocol combines pulses of 125mg of methyl-prednisolone with each fortnightly pulse of cyclophosphamide and prednisone ≤30mg/day with tapering over 12-14weeks until 2.5-5mg/day 5
• A protocol using medium doses of prednisone to treat lupus nephritis, combined with methyl-prednisolone pulses, hydroxychloroquine, and immunosuppressive drugs, usually cyclophosphamide, has been shown to be effective and safe 6

Dosage and Efficacy

• The dose and duration of corticosteroids required for control of lupus nephritis and CNS lupus have never been tested in a randomized trial design, so current recommendations are based on observation and expert opinion 2
• A lower dose regimen of MMF and steroids may result in better long-term safety, including a reduction in lymphoproliferative disorders, skin cancers, and steroid-related side effects, without compromising efficacy 3
• Repeated pulses of methyl-prednisolone with reduced doses of prednisone have been shown to improve the outcome of class III, IV, and V lupus nephritis 5