Past and present of childhood kidney disease: advances in treatment based on the pathogenesis of pediatric IgA nephropathy

Abstract

IgA nephropathy (IgAN) is more frequently found in chronic glomerulonephritis (CGN) in childhood. Recently, it is reported that the pathogenesis of IgAN may be associated with defective IgA1 forms circulating or in situ immune complexes. In the presence of the genetic backgrounds, the defective IgA1 immune complex produced by T cells and B cells activated by antigen stimulation is deposited in the glomerulus and these immune complexes induce inflammation through activation of complement, macrophages and mesangial cells, and glomerular damage progresses. The prognosis of IgA nephropathy varies from normalization of urinary findings to deterioration of proteinuria and deterioration of renal function. To control these inflammations, each useful treatment was chosen according to the severity of IgAN. For children with severe IgAN, the long-term prognosis improved by multi-drug combination therapy including steroid and immunosuppressive drugs or tonsillectomy steroid plus therapy. In the future, it is expected that a treatment method with less side effects will be established.