2 Minor glomerular abnormalities 216 25.1 408 37.5 624 32.0 Mesangial proliferative glomerulonephritis 167 19.4 86 7.9 253 13.0 Focal segmental glomerulosclerosis 113 13.1 149 13.7 262 13.4 Membranoproliferative glomerulonephritis (types I and III) 48 5.6 51 4.7 99 5.1 Crescentic and necrotizing glomerulonephritis 19 2.2 18 1.7 37 1.9 Endocapillary

proliferative glomerulonephritis 8 0.9 24 2.2 32 1.6 Chronic interstitial nephritis 7 0.8 3 0.3 10 0.5 Sclerosing glomerulonephritis 7 0.8 3 0.3 10 0.5 Nephrosclerosis 5 0.6 7 0.6 12 0.6 Acute interstitial nephritis 1 0.1 0 – 1 0.1 Acute tubular necrosis 0 – 1 0.1 1 0.1 Others 11 1.3 9 0.8 20 1.0 Total 861 PF01367338 100.0 1,089 100.0 1,950 100.0 In the patients with nephrotic syndrome as classified by the clinical diagnosis, primary glomerular disease other than IgAN was the predominant diagnosis in both 2009 and 2010, followed by diabetic nephropathy, Liproxstatin-1 manufacturer which was the same order as in 2007 and 2008 (Table 9). In 2010, minor glomerular abnormalities were the leading diagnosis, followed by MN, FSGS, and MPGN (types I and III) (Table 10). Table 9 The frequency of pathological diagnoses as classified by pathogenesis in nephrotic syndrome in native kidneys in J-RBR 2009 and 2010 Classification 2009 2010 Total n % n % n % Primary glomerular disease (except IgA nephropathy) 442 62.3 696 CYTH4 66.7 1,138 64.9 Diabetic nephropathy 85 12.0 78 7.5 163 9.3 IgA nephropathy 30 4.2 36 3.5 66 3.8 Lupus nephritis 30 4.2 58 5.6 88 5.0 Amyloid nephropathy 27 3.8 41 3.9 68 3.9 Infection-related nephropathy 6 0.8 7 0.7 13 0.7 Hypertensive nephrosclerosis 6 0.8 10 0.9 16 0.9 Purpura nephritis 4 0.6 8 0.8 12 0.7 Alport syndrome 3 0.4 0 – 3 0.2 Thrombotic microangiopathy 1 0.1 1 0.1 2 0.1 PR3-ANCA positive nephritis 1 0.1 0 – 1 0.1 MPO-ANCA positive nephritis 1 0.1 2 0.2 3 0.2 Others 74 10.4 106 10.2 180 10.3 Total 710 100.0

1,043 100.0 1,753 100.0 MPO myeloperoxidase, ANCA anti-neutrophil cytoplasmic antibody, PR3 proteinase 3 Table 10 The frequency of pathological diagnoses as classified by histopathology in primary glomerular disease except IgA nephropathy in nephrotic syndrome in native kidneys in J-RBR 2009 and 2010 Classification 2009 2010 Total n % n % n % Membranous nephropathy 178 40.3 227 32.6 405 35.6 Minor glomerular abnormalities 172 38.9 348 50.0 520 45.7 Focal segmental glomerulosclerosis 47 10.6 82 11.8 129 11.3 Membranoproliferative glomerulonephritis (types I and III) 25 5.7 18 2.6 43 3.8 Mesangial proliferative glomerulonephritis 11 2.5 13 1.9 24 2.1 Crescentic and necrotizing glomerulonephritis 2 0.5 2 0.3 4 0.4 Sclerosing glomerulonephritis 2 0.5 0 – 2 0.