124 IgA 3 3 IgA IgA 16 IgA H-Grade25 %25 % 50 % 50 % 75 %75 % g C-Grade 0.5 g egfr 60 ml min 1.73 m 2 C-Grade 0.5 g egfr 60 ml min

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1 IgA IgA 3 IgA 3 IgA IgA 1995 IgA 2002 IgA 2 IgA IgA IgA IgA 3 Clinical guides for immunoglobulin A IgA nephropathy in Japan, third version

2 124 IgA 3 3 IgA IgA 16 IgA H-Grade25 %25 % 50 % 50 % 75 %75 % g C-Grade 0.5 g egfr 60 ml min 1.73 m 2 C-Grade 0.5 g egfr 60 ml min 1.73 m 2 C-Grade IgA 7 IgA IgA 1 IgA J. Berger N. Hinglais IgA IgG % 20 % IgA chance proteinuria and or hematuria IgA %

3 125 3 IgA IgA C 3 IgA IgA1 J 2 IgA IgA 1 IgA 2 3 IgA EB 50 % IgA IgA IgA1 IgA mast cell IgA IgA IgA 2 1 IgA IgA 1 6 IgA 2 IgA * IgA 4 16 IgA IgA 2 IgA 2 *

4 126 IgA 3 1 IgA % A B 2 C 3 A 3 B IgA 315 mg dl 4 A 4 B IgA C 1 2 A 2 B 3 B HPF 2 3 IgA 350 mg dl IgA 4 IgA 3 IgA 4 * * IgA International IgA Nephropathy Network Renal Pathology Society 2009 IgA 8,9

5 127 * %OR split system 3 % AND lumped system % 30 % AND %OR 10 IgA IgA 2 1 % 10 % % % 50 % 11,12 2 8,9 11,12 * 2 IgA 2 7 8,9 50 % p 0.01 * IgA 3 1 A G 13 3 lumped system * 3 10 % 30 % OR split system 1 Cr Wakai 15

6 128 IgA 3 2IgA diffuse50 % focal50 % global 50 % segmental 50 % endocapillary hypercellularity karyorrhexis necrosis 2 GBM duplication increased mesangial matrix 2 2 sclerosis adhesion* segmental sclerosis global sclerosis collapsed ischemic glomerulus * extracapillary lesions extracapillary cellular proliferation or cellular crescent3 50 % % 10 % % %50 % extracapillary fibrocellular proliferation or fibrocellular crescent 50 % 90 %% 10 % % %50 % extracapillary fibrous proliferation or fibrous crescent90 % * 10 %% % %50 % mesangial hypercellularity normal 3 mild 4 5 moderate 6 7 severe IgA cellular crescent fibrocellular crescent global sclerosis segmental sclerosis fibrous crescent 5 % 10,16

7 129 1 ABC D E FG % A A C C % A A C C % A A C C 75 % A A C C A C 25 % 50 % 75 % H-Grade25 % H-Grade25 % 50 % H-Grade50 % 75 % H-Grade 75 % 4 acute lesion A chronic lesion C A A C C 4 12,17,18 IgA

8 130 IgA 3 9 Spearman s rank correlation R p IgA IgA 2 g 4 Jaffé egfr 100 HPF egfr 0.5 gegfr 60 ml min 1.73 m g egfr 60 ml min 1.73 m 2 60 ml min 1.73 m 2 egfr 0.5 g 0.5 g 0.5 g egfr 60 ml min 1.73 m 2 60 ml min 1.73 m g egfr ml min 1.73 m % OR % OR % OR % OR % OR % OR 28.4 H-Grade % OR % OR % OR 130 %OR vs H- Grade 0.5 g C-Grade 0.5 g egfr 60 ml min 1.73 m 2 C-Grade 0.5 g egfr 60 ml min 1.73 m 2 C-Grade 3 17,18 5 IgA H- GradeC-Grade 6 C-Grade H-Grade % IgA H- Grade C-Grade

9 131 7 IgA H-Grade % % % % % % 70 % RA % % % % RA % RA % % % RA 4 RA J-KDR IgA Japan IgA nephropathy cohort study J-IGACS IgA IgA 1986 Kobayashi ,21,24,25 IgA Hotta IgA fish oil 32

10 132 IgA 3 8 IgA IgA CKD CKD CKD IgA 9 A 33 1 B egfr 1 34 C kcal kg 35 33,34 1 A B g kg 36,37 C A B g kg 36 6 g 36,37 C 2, gegfr 60 ml min 1.73 m A B g kg 36 6 g 36,37 35,36 C 2, 3 egfr 60 ml min 1.73 m A 38 B 6 g 36, g kg 36 35,36 C 9 1 m 2 22kg 2 39,

11 133 9 IgA IgA g egfr 60 ml min 1.73 m 2 41, mg 2 41, mg RCT 23 egfr 60 ml min 1.73 m 2 41, mg dl g 1 g , mg dl , mg dl mmhg 1 g mmhg mg dl IgA * IgA 0.5 g IgA IgA 3 CKD 50

12 134 IgA 3 IgA 10 3 IgA Haas M. Histologic subclassification of IgA nephropathy a clinico pathologic study of 244 cases. Am J Kidney Dis Lee KSM. Prognostic indicators of progressive renal disease in IgA nephropathy Emergence of a new histologic grading system. Am J Kidney Dis Manno C, Strippoli GF, D Altri C, et al. A novel simpler histological classification for renal survival in IgA nephropathy a retrospective study. Am J Kidney Dis Shigematsu H. Histological grading and staging of IgA nephropathy. Pathol Int Lee HS, Lee MS, Lee SM, et al. Histological grading of IgA nephropathy predicting renal outcome revisiting H. S. Lee s glomerular grading system. Nephrol Dial Transplant Katafuchi R, Kiyoshi Y, Oh Y et al. Glomerular score as a prognosticator in IgA nephropathy its usefulness and limitation. Clin Nephrol Tomino Y, Sakai H, Special Study Group IgA Nephropathy on Progressive Glomerular Disease. Clinical guidelines for immunoglobulin A IgA nephropathy in Japan, second version. Clin Exp Nephrol A Working Group of the International IgA Nephropathy Network and the Renal Pathology Society Cattran DC, Coppo R, Cook HT, et al. The Oxford classification of IgA nephropathy rationale, clinicopathological correlations, and classification. Kidney Int A Working Group of the International IgA Nephropathy Network and the Renal Pathology Society Roberts ISD, Cook T, Troyanov S, et al. The Oxford classification of IgA Nephropathy Pathology definitions, correlations and reproducibility. Kidney Int IgA AND OR IgA IgA IgA Annual Review IgA Wakai K, Kawamura T, Endoh M, et al. A scoring system to predict renal outcome in IgA nephropathy from a nationwide prospective study. Nephrol Dial Transplant IgA IgA IgA IgA Annual Review Kobayashi Y, Fujii K, Hiki Y, et al. Steroid therapy in IgA nephropathy A prospective pilot study in moderate proteinuric cases. Q J Med IgA Pozzi C, Bolasco P, Fogazzi G, et al. Corticosteroids in IgA nephropathy a randomized controlled trial. Lancet Shoji T, Nakanishi I, Suzuki A, et al. Early treatment with corticosteroids ameliorates proteinuria, proliferative lesions, and mesangial phenotypic modulation in adult diffuse proliferative IgA nephropathy. Am J Kidney Dis Katafuchi R, Ikeda K, Mizumasa T, et al. Controlled, prospective trial of steroid treatment in IgA nephropathy A limitation of low-dose prednisolone therapy. Am J Kidney Dis Pozzi C, Andrulli S, Del Vecchio L, et al. Corticosteroid effectiveness in IgA nephropathy long-term results of a randomized, controlled trial. Am Soc Nephrol Katafuchi R, Ninomiya T, Mizumasa T, et al. The improvement of renal survival with steroid pulse therapy in IgA nephropathy. Nephrol Dial Transplant Hotta O, Miyazaki M, Furuta T, et al. Tonsillectomy and steroid pulse therapy significantly impact on clinical remission in patients with IgA nephropathy. Am J Kidney Dis Xie Y, Nishi S, Ueno M, et al. The efficacy of tonsillectomy

13 135 on long-term renal survival in patients with IgA nephropathy. Kidney Int Komatsu H, Fujimoto S, Hara S, et al. Effect of tonsillectomy plus steroid pulse therapy on clinical remission of IgA nephropathy a controlled study. Clin J Am Soc Nephrol IgA Yoshikawa N, Ito H, Sakai T, The Japanese Pediatric IgA Nephropathy Treatment Study Group. A controlled trial of combined therapy for newly diagnosed severe childhood IgA nephropathy. J Am Soc Nephrol Ballardie FW, Roberts IS. Controlled prospective trial of prednisolone and cytotoxics in progressive IgA nephropathy. J Am Soc Nephrol Donadio JV Jr, Bergstralh EJ, Offord KP, Spencer DC, Holley KE. A controlled trial of fish oil in IgA nephropathy. Mayo Nephrology Collaborative Group. N Engl J Med CKD CKD CKD CKD CKD CKD CKD CKD Kobayashi Y, Hiki Y, Kokubo T, et al. Steroid therapy during the early stage of progressive IgA nephropathy. A 10 year follow-up study. Nephron Tomino Y, Suzuki H, Horikoshi S, et al. Multicenter trial of adrenocorticosteroids in Japanese patients with IgA nephropathy results of the special study group IgA nephropathy on progressive glomerular disease, Ministry of Health, Labor and Welfare of Japan. Curr Top Steroid Res Moriyama T, Honda K, Nitta K, et al. The effectiveness of steroid therapy for patients with advanced IgA nephropathy and impaired renal function. Clin Exp Nephrol Tamura S, Ueki K, Ideura H, et al. Corticosteroid therapy in patients with IgA nephropathy and impaired renal function. Clin Nephrol Sato M, Hotta O, Tomioka S, et al. Cohort study of advanced IgA nephropathy efficacy and limitations of corticosteroids with tonsillectomy. Nephron Clin Pract c IgA CKD Russo D, Minutolo R, Pisani A, et al. Coadministration of losartan and enalapril exerts additive antiproteinuric effect in IgA nephropathy. Am J Kidey Dis Tomino Y, Kawamura T, Kimura K, et al. Antiproteinuric effect of olmesartan in patients with IgA nephropathy. J Nephrol Lee GSL, Choong HL, Chiang GSC, et al. Three-year randomized controlled trial of dipyridamole and low-dose warfarin in patients with IgA nephropathy and renal impairment. Nephrology Ishii T, Kawamura T, Tsuboi N, et al. Prospective trial of combined therapy with heparin warfarin and renin-angiotensin system inhibitors in progressive IgA nephropathy. Contrib Nephrol

9 IgA 腎症 ステートメント 1 総論 IgA 腎症は発症後 20 年以内に約 40% が末期腎不全に至る疾患であり 1), 予後不良因子 ( 収縮期高血圧, 高度蛋白尿, 血清 Cr 高値, 障害度の高い腎生検所見 ) を有する患者では, 早期より積極的な治療が推奨される 2-4). 2 RA

9 IgA 腎症 ステートメント 1 総論 IgA 腎症は発症後 20 年以内に約 40% が末期腎不全に至る疾患であり 1), 予後不良因子 ( 収縮期高血圧, 高度蛋白尿, 血清 Cr 高値, 障害度の高い腎生検所見 ) を有する患者では, 早期より積極的な治療が推奨される 2-4). 2 RA 9 IgA 腎症 ステートメント 1 総論 IgA 腎症は発症後 20 年以内に約 40% が末期腎不全に至る疾患であり 1), 予後不良因子 ( 収縮期高血圧, 高度蛋白尿, 血清 Cr 高値, 障害度の高い腎生検所見 ) を有する患者では, 早期より積極的な治療が推奨される 2-4). 2 RA 系阻害薬 ACEI は蛋白尿を減少させ, 腎機能障害進行を抑制することから, 高血圧を合併した IgA

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