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雑誌文献

臨床検査68巻1号

2024年01月発行

文献概要

今月の特集2 補体をめぐる話題 注目の補体異常症

C3腎症

著者: 金恒秀1 水野正司1

所属機関: 1名古屋大学大学院医学系研究科病態内科学腎臓内科

ページ範囲:P.88 - P.92

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●C3腎症は非典型溶血性尿毒症症候群(aHUS)と並ぶ代表的な補体異常による腎臓病である.

●腎生検病理組織像の蛍光染色において,C3がその他の染色と比較して有意に染色されていることから診断され,電子顕微鏡の所見によって,C3腎炎とDDD(dense deposit disease)にさらに分類される.

●既存の薬剤による治療効果は限定的であるが,現在さまざまな抗補体薬による治験が行われており,その効果が期待されている.

参考文献

1)Pickering MC, D'Agati VD, Nester CM, et al:C3 glomerulopathy: consensus report. Kidney Int 84:1079-1089,2013
2)Goodship TH, Cook HT, Fakhouri F, et al:Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. Kidney Int 91:539-551,2017
3)Rovin BH, Caster DJ, Cattran DC, et al:Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int 95:281-295,2019
4)Bomback AS, Appel GB:Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol 8:634-642,2012
5)Servais A, Noël LH, Roumenina LT, et al:Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 82:454-464,2012
6)Heiderscheit AK, Hauer JJ, Smith RJH:C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease. Am J Med Genet C Semin Med Genet 190:344-357,2022
7)Banks RA, May S, Wallington T:Acute renal failure in dense deposit disease: recovery after plasmapheresis. Br Med J (Clin Res Ed) 284:1874-1875,1982
8)Krmar RT, Holtbäck U, Linné T, et al:Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange. Clin Nephrol 75(Suppl 1):4-10,2011
9)Rabasco C, Cavero T, Román E, et al:Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int 88:1153-1160,2015
10)Caravaca-Fontán F, Díaz-Encarnación MM, Lucientes L, et al:Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease. Clin J Am Soc Nephrol 15:1287-1298,2020
11)Rudnicki M:Rituximab for Treatment of Membranoproliferative Glomerulonephritis and C3 Glomerulopathies. Biomed Res Int 2017:2180508,2017
12)Bomback AS, Smith RJ, Barile GR, et al:Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 7:748-756,2012
13)Ruggenenti P, Daina E, Gennarini A, et al:C5 Convertase Blockade in Membranoproliferative Glomerulonephritis: A Single-Arm Clinical Trial. Am J Kidney Dis 74:224-238,2019
14)Nester C, Appel GB, Bomback AS, et al:Clinical Outcomes of Patients with C3G or IC-MPGN Treated with the Factor D Inhibitor Danicopan: Final Results from Two Phase 2 Studies. Am J Nephrol 53:687-700,2022
15)Bomback AS, Kavanagh D, Vivarelli M, et al:Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial. Kidney Int Rep 7:2150-2159,2022

掲載誌情報

出版社:株式会社医学書院

電子版ISSN:1882-1367

印刷版ISSN:0485-1420

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