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“Introduction Immunoglobulin A nephropathy (IgAN) was first described by Berger et al. . Approximately 40% of IgAN patients develop renal failure within 20 years of diagnosis, and the long-term prognosis is poor . Pozzi et al.  GDC-0449 cost reported that corticosteroid therapy for IgAN exerted a renoprotective effect, but that relapse of proteinuria was observed in a relatively large number of patients after treatment. This report also suggested that complete remission (CR) cannot be achieved without preventing continuous tissue deposition of IgA. Focal infection of the palatine tonsils or other mucosal sites causes immune abnormalities, leading Regorafenib nmr to sugar-chain incompleteness in IgA1, which is then overproduced and deposited in renal glomeruli . In Japan, high rates of
CR have been reported in patients with early IgAN after bilateral palatine tonsillectomy and steroid pulse therapy [5, 6]. In some patients, however, steroid-associated adverse events have occurred in a dose-dependent manner, learn more necessitating dose reduction. An increase in the number of sclerotic glomeruli as well as in the degree of interstitial fibrosis due to steroid therapy has also been reported in patients with low glomerular filtration rates (GFRs) . Mizoribine (MZR) is an immunosuppressive agent used for the treatment of nephrotic syndrome caused by primary glomerulonephritis. A decrease in the intensity of IgA staining in glomerular mesangial areas, as well as a decrease in the number of B cells Erythromycin and IgA-bearing B cells, has been demonstrated in a MZR-treated animal model of IgAN . In another study involving 34 children with diffuse IgAN who received steroid pulse therapy in combination with MZR, there was a significant
decrease in the degree of IgA deposition and infiltration of the glomeruli by CD68-positive cells and alpha-smooth muscle actin-positive cells, and consequently a decrease in the extent of tissue damage . Other reports have also indicated that MZR ameliorates glomerular sclerosis and tubulointerstitial fibrosis [10, 11]. To reduce the total dose of steroids, since 2004 we have been using MZR for IgAN in combination with tonsillectomy and steroid pulse therapy. Specifically, patients receive one course of steroid pulse therapy instead of the current three courses and postoperative oral steroid therapy for 7 months instead of 11 months, in combination with MZR. In the present study, data from 42 patients followed up for at least 24 months were used to determine the rate of CR (assessed by urinalysis), the treatment efficacy in protecting against renal function deterioration, and the safety of the therapy.