What is Systemic Lupus Erythematosus (SLE)?

From the Guidelines

The management of Euro lupus should prioritize treatment with a focus on achieving remission or low disease activity, and preventing flares, as recommended by the 2023 update of the EULAR recommendations 1. The Euro-Lupus protocol is a treatment approach that has been studied in the context of lupus nephritis, and it typically consists of a lower-dose cyclophosphamide regimen.

• The protocol involves 500 mg of intravenous cyclophosphamide every two weeks for a total of six doses (3 months), followed by maintenance therapy with azathioprine or mycophenolate mofetil.
• This regimen is often accompanied by corticosteroids, usually starting with prednisone at 0.5-1 mg/kg/day, which is then tapered gradually.
• The use of hydroxychloroquine is recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight, as it has been shown to be beneficial in reducing disease activity and preventing flares 1.
• In patients with extrarenal disease, anifrolumab and belimumab have been shown to be superior to standard of care treatment in high-quality randomised controlled trials 1.
• For patients with lupus nephritis, combination treatments with belimumab or voclosporin have been found to be more effective than standard of care in achieving remission and low disease activity 1.
• Regular monitoring of kidney function, urinalysis, and blood counts is essential during treatment to assess response and detect potential toxicities early.
• The goal of treatment should be to achieve remission or low disease activity, as this has been associated with a lower risk of adverse outcomes, including flares and organ damage 1.

From the Research

Euro Lupus

• Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that requires treatment with synthetic drugs to ameliorate symptoms and positively influence outcome 2.
• Hydroxychloroquine (HCQ) is an effective treatment for SLE, and it has been shown to reduce the risk of flares, allow the reduction of the dosage of steroids, reduce organ damage, and prevent the thrombotic effects of anti-phospholipid antibodies 3.
• The daily and weight-adjusted dosages of HCQ are important considerations in the treatment of SLE, with a mean weight-adjusted dose of 3.1 mg/kg/day being commonly used 4.
• HCQ has been shown to have a favorable impact on long-term outcomes such as damage accrual and mortality in SLE, and it is now the mainstay long-term treatment in SLE, recommended by current guidelines in all patients unless contraindications or side effects 5.
• Other treatments for SLE include mycophenolate mofetil, azathioprine, and biologic agents such as belimumab, which have been shown to be effective in reducing disease activity and improving outcomes 2, 6.

Treatment Options

• HCQ is considered an anchor drug in SLE due to its multiple beneficial effects, and it should be considered as a first-line treatment for patients with mild, moderate, and severe lupus erythematosus 2, 3.
• Azathioprine and mycophenolate mofetil are also effective treatments for SLE, and they should be considered as additional immunosuppressants for patients who do not respond to HCQ or require higher dosages of glucocorticoids 2.
• Biologic agents such as belimumab have been shown to be effective in reducing disease activity and improving outcomes in patients with SLE, and they should be considered as an option for patients who do not respond to traditional treatments 6.

Dosage and Safety

• The daily dose of HCQ associated with the best compromise between efficacy and safety is a matter of debate, with a daily dose of ≤5 mg/kg/day actual body weight currently agreed upon 5.
• The use of HCQ at stable doses of 200 mg/day (or 3.0-3.5 mg/kg/day) as the background therapy in patients with SLE has been shown to be effective in achieving prolonged remission 4.
• The safety of HCQ has been well established, with a low risk of side effects and a favorable impact on long-term outcomes such as damage accrual and mortality in SLE 3, 5.