An Update on Treatment and Outcome of Lupus Nephritis in Children

Abstract

Lupus nephritis (LN) is the most common manifestation of systemic lupus erythematosus (SLE) in children to determine the course and outcome of the disease. Although its prognosis has improved in recent decades, varying degrees of chronic renal impairment, including end-stage renal disease (ESRD), can still develop. Early and adequate treatment in LN protects the kidneys from developing chronic damage. Treatment should be based on the clinical severity of the disease and renal histology. Although there are still many controversies on the best treatment of pediatric LN, corticosteroids (CS) are still the first-line drugs used for induction therapy. Less severe forms of LN do not require specific therapy, apart from the treatment of underlying SLE itself, but focal or diffuse proliferative glomerulonephritis requires more aggressive therapy with different combinations of other immunosuppressive drugs. Induction pro tocols in most centers mainly included intravenous cyclophosphamide (CYC) pulse therapies with promising initial results, although more recent studies have been less encouraging. Oral mycophenolate mofetil (MMF) was shown to be at least equally effective as CYC for induction and maintenance therapies and has now become more commonly utilized in pediatric patients. There have also been increasing reports about successful B-lymphocyte depletion therapy with rituximab (RTX) in recent years. Mizoribine, azathioprine, cyclosporine A, and hydroxychloroquine are other therapeutic agents that are used in many centers for induction and maintenance therapies. Plasma exchange and stem cell transplantation can be used in very severe and refractory cases of SLE. New treatment approaches are still under investigation.