What is the treatment for a systemic lupus erythematosus (SLE) flare?

From the Guidelines

To treat a lupus flare, the primary goal is to aim at remission or low disease activity and prevention of flares in all organs, maintained with the lowest possible dose of glucocorticoids, as recommended by the 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus 1. When managing a lupus flare, it's essential to consider the severity of organ involvement and adjust ongoing therapies, such as glucocorticoids and immunomodulating agents, to higher doses, switch, or add new therapies as needed 1. Some key considerations for treatment include:

• Using hydroxychloroquine (HCQ) for all patients with SLE, unless contraindicated, at a dose not exceeding 5 mg/kg/real BW 1
• Utilizing glucocorticoids (GC) at doses and routes of administration that depend on the type and severity of organ involvement, with the goal of minimizing chronic maintenance treatment to less than 7.5 mg/day (prednisone equivalent) and, when possible, withdrawing GC 1
• Considering immunomodulating/immunosuppressive agents such as methotrexate, azathioprine, or mycophenolate for patients not responding to HCQ or GC, or for those with organ-threatening disease 1
• Adding biologics like belimumab for patients with inadequate response to standard-of-care, or rituximab for organ-threatening disease refractory or with intolerance/contraindications to standard immunosuppressive agents 1 It's crucial to tailor treatment to the individual patient's needs, taking into account the specific manifestations of their lupus flare, such as skin, neuropsychiatric, hematological, or renal disease, and adjusting treatment accordingly 1. For example, in cases of lupus thrombocytopenia, high-dose GC and/or intravenous immunoglobulin G may be used for acute treatment, while immunosuppressive/GC-sparing agents like mycophenolate or azathioprine can be used for maintenance of response 1. In renal disease, early recognition and diagnostic renal biopsy are essential, with mycophenolate or low-dose intravenous cyclophosphamide recommended as initial treatment, and mycophenolate or azathioprine for maintenance therapy 1. Overall, the treatment of a lupus flare should be guided by the principles of minimizing disease activity, preventing flares, and maintaining the lowest possible dose of glucocorticoids, while tailoring treatment to the individual patient's needs and specific disease manifestations 1.

From the FDA Drug Label

BENLYSTA is a B-lymphocyte stimulator (BLyS)-specific inhibitor indicated for the treatment of: • patients 5 years of age and older with active systemic lupus erythematosus (SLE) who are receiving standard therapy; • patients 5 years of age and older with active lupus nephritis who are receiving standard therapy DOSAGE AND ADMINISTRATION • Intravenous Dosage for Adult and Pediatric Patients with SLE or Lupus Nephritis:−10 mg/kg at 2‑week intervals for the first 3 doses and at 4‑week intervals thereafter.

To treat a lupus flare, belimumab (BENLYSTA) can be used as part of the treatment plan. The recommended dosage is:

• Intravenous dosage: 10 mg/kg at 2-week intervals for the first 3 doses and at 4-week intervals thereafter.
• Subcutaneous dosage: varies depending on the patient's weight and condition (SLE or lupus nephritis). It is essential to note that belimumab should be used in conjunction with standard therapy for SLE or lupus nephritis. Patients should be closely monitored for signs of infection, allergic reactions, and mental health problems while receiving belimumab 2.

From the Research

Treatment Options for Lupus Flare

• Nonsteroidal anti-inflammatory drugs (NSAIDs) are commonly used to treat musculoskeletal symptoms, serositis, and headache in patients with systemic lupus erythematosus (SLE) 3
• Traditional treatments for SLE include NSAIDs and antimalarial agents, which are the first-line therapies for mild SLE 4
• Glucocorticoids and cytotoxic or immunosuppressive agents, such as azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporine, and methotrexate, are used for SLE with organ involvement 4
• Targeted immunotherapies, such as the anti-BAFF antibody belimumab, have been approved as an add-on therapy for patients who have active disease despite receiving standard therapy 4, 5
• B-cell modulation, T-cell regulation, and cytokine inhibition are potential therapeutic targets for SLE, with belimumab being the only approved agent for lupus treatment to date 5
• Non-steroidal anti-inflammatory drugs, such as hydroxychloroquine, and glucocorticoids are also used to treat SLE 5, 6

Immunosuppressive Drugs and Corticosteroids

• A systematic review and network meta-analysis found that cyclophosphamide, mycophenolate mofetil, and azathioprine were associated with a lower odds of renal relapse/flare compared to prednisone 7
• Tacrolimus was more likely than cyclophosphamide to achieve renal remission/response, while cyclosporine was more likely than cyclophosphamide and mycophenolate mofetil to achieve renal remission/response 7
• Cyclophosphamide was associated with a higher risk of amenorrhea/ovarian failure and cytopenia compared to mycophenolate mofetil and prednisone 7

Management Goals

• Maximization of clinical response and avoidance of side effects are important management goals for patients with SLE 6
• Different dosage regimens, such as intravenous methylprednisolone pulse therapy, and adjunctive agents, such as cyclophosphamide, are of continued interest in severe and potentially life-threatening disease 6