Lupusnephritis im Kindes- und Jugendalter

Lupus nephritis (LN) occurs in 60–80% of patients with juvenile systemic lupus erythematosus (jSLE) and is decisive for the morbidity and mortality. A timely kidney biopsy should be performed in the presence of significant proteinuria and/or impaired renal function with or without hematuria to determine the type and extent of renal involvement. The histological classification of LN according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) is fundamental to the treatment decision. The primary therapeutic goal is to achieve and maintain remission and to minimize drug toxicity. In patients with proliferative LN (classes III and IV) and/or class V LN, an intensified immunosuppression should be given as induction therapy for 3–6 months followed by maintenance therapy for at least 3 years. New treatment approaches focus on targeted interventions in specific immunoreactive cells, cytokines and signalling pathways. Examples include belimumab and obinutuzumab, which have shown promising results in adults with LN but valid data on the efficacy and safety in children with LN are still lacking. The European Society of Pediatric Nephrology (ESPN) and the European Rare Kidney Diseases Reference Network (ERKNet) have initiated a prospective multinational registry for children and adolescents with LN to study the clinical course and impact of drug treatment in these patients and to improve the treatment and long-term outcome.