2016 Volume 29 Issue 2 Pages 94-101
IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide both in children and adults. Japanese annual school urinary screening program can determine the onset time of IgAN within one year, and enables us to conduct clinical trials in patients with IgAN early during the course of disease. Based on the results of clinical trials, the Japanese Society for Pediatric Nephrology has developed “Guidelines for the treatment of childhood IgAN.” In these guidelines, the disease severity has been divided into two categories, i.e., mild and severe IgAN, and according to the severity, treatments were proposed. Treatment of children with severe IgAN showing diffuse mesangial proliferation with prednisolone, azathioprine, heparin-warfarin, and dipyridamole for 2 years early in the course of disease prevents immunologic renal injury and progression of the disease, and not only ameliorates the activity of the acute phase of nephritis but also improves the long-term outcome of severe childhood IgAN. Lisinopril for 2 years is recommended for mild cases with IgAN. As to the role of tonsillectomy in the treatment of IgAN employed mainly in adults, at present studies provide conflicting data. Therefore, it cannot be recommended for widespread use for the treatment of IgAN patients, especially for children with IgAN.