The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative has formulated evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis, which were recently published in the Annals of the Rheumatic Diseases.

Recommendations were developed using the European League Against Rheumatism (EULAR) standard operating procedures. Overall, 6 recommendations regarding diagnosis and 20 recommendations on treatment choices and goals were accepted with ≥93% agreement among a group of European experts on childhood-onset systemic lupus erythematosus, including pediatric nephrologists. The treatment goal of pediatric lupus nephritis is complete renal response.

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The treatment of patients with class I lupus nephritis should be guided mainly by other symptoms. Patients with class II lupus nephritis should be treated initially with low-dose prednisone and a disease-modifying antirheumatic drug should be added only after 3 months of persistent proteinuria or prednisone dependency. In those with class III/IV lupus nephritis, induction therapy should be administered with mycophenolate mofetil (MMF) or intravenous (IV) cyclophosphamide plus corticosteroids. Maintenance treatment in patients with class III/IV lupus nephritis should include MMF or azathioprine for at least 3 years. In patients with pure class V lupus nephritis, MMF plus low-dose prednisone can be used as induction therapy and MMF can be given as maintenance treatment.

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As a general rule, in patients with childhood-onset lupus nephritis who do not respond to treatment or in whom disease flares develop, medication noncompliance should be the first issue explored. If a patient does not respond to treatment within 3 months of induction therapy, the primary induction agent should be changed. In patients with persistent active or refractory cases of class III or IV lupus nephritis, with or without class V lupus nephritis, treatment should be switched to another pharmacologic agent. For example, when a patient is treated with MMF, the agent should be switched to rituximab or IV cyclophosphamide.

The goal of these evidence-based guidelines for the diagnosis and treatment of lupus nephritis is to provide the tools for uniform and high-quality care for all pediatric patients with the disease.

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Groot N, de Graeff N, Marks SD, et al. European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative. Ann Rheum Dis. 2017;76(12):1965-1973.