What is the acute management of a systemic lupus erythematosus (SLE) flare in the emergency department (ED)?

From the Guidelines

The acute management of a lupus flare in the emergency department should prioritize rapid assessment and targeted treatment based on the severity and organ systems involved, with high-dose corticosteroids as the mainstay of treatment for mild to moderate flares, as recommended by the 2019 EULAR guidelines 1. The initial management should include a thorough evaluation with complete blood count, comprehensive metabolic panel, urinalysis, and inflammatory markers (ESR, CRP) to assess disease activity.

• For mild to moderate flares, high-dose corticosteroids are recommended, typically prednisone 0.5-1 mg/kg/day or methylprednisolone 125-1000 mg IV daily for 1-3 days, as stated in the guidelines 1.
• Hydroxychloroquine should be continued or initiated at 200-400 mg daily, as it reduces flare frequency, according to the guidelines 1. Some key points to consider in the management of lupus flares include:
• The use of pulses of intravenous methylprednisolone (usually 250–1000 mg per day, for 1–3 days) to provide an immediate therapeutic effect and enable the use of lower starting doses of oral glucocorticoids, as recommended by the guidelines 1.
• The importance of minimizing chronic maintenance treatment with glucocorticoids to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawing them, as stated in the guidelines 1.
• The consideration of immunomodulating/immunosuppressive agents, such as methotrexate, azathioprine, or mycophenolate, for patients not responding to hydroxychloroquine or glucocorticoids, or for those with organ-threatening disease, as recommended by the guidelines 1. For severe flares with major organ involvement, more aggressive therapy is warranted, with methylprednisolone 1000 mg IV daily for 3 days, followed by oral prednisone, and consideration of cyclophosphamide, mycophenolate mofetil, or rituximab for life-threatening manifestations, as suggested by the guidelines 1.

From the Research

Acute Management of Lupus Flare in the Emergency Department

• The management of lupus flare in the emergency department involves the use of immunosuppressive therapies, such as cyclophosphamide and methylprednisolone, to reduce disease activity and prevent organ damage 2, 3, 4, 5.
• Pulse steroid therapy, including methylprednisolone, is commonly used in the emergency department to manage lupus flare, with 32.5% of patients with an initial diagnosis of SLE receiving pulse steroid therapy 6.
• The combination of pulse cyclophosphamide and methylprednisolone has been shown to improve long-term renal outcome without adding toxicity in patients with lupus nephritis 5.
• Rituximab, a biologic agent, has also been used in combination with cyclophosphamide and methylprednisolone to induce remission in severe cases of SLE with nephritis, with minimal side effects 4.
• Early recognition and appropriate management of SLE-related conditions and other comorbidities in the emergency department are crucial to improve clinical outcomes and reduce mortality 6.

Treatment Options

• Cyclophosphamide: remains the 'gold standard' treatment for severe organ-threatening SLE, especially renal and central nervous system lupus 2.
• Methylprednisolone: used in combination with cyclophosphamide to improve long-term renal outcome without adding toxicity in patients with lupus nephritis 5.
• Rituximab: used in combination with cyclophosphamide and methylprednisolone to induce remission in severe cases of SLE with nephritis 4.
• Pulse steroid therapy: commonly used in the emergency department to manage lupus flare 6.

Clinical Outcomes

• The rate of mortality was 6.8% in the 6-month follow-up period, with all deaths occurring in patients with disease-established SLE 6.
• SLEDAI scores were significantly decreased after 6 months of therapy 6.
• Complete renal remission rates were 69% and 86% at 6 and 12 months, respectively, in patients treated with the Lupus-Cruces nephritis protocol 3.