C3 glomerulopati hastalığı
Year 2018,
, 1 - 8, 20.03.2018
Can Huzmeli
,
Mehmet Hadi Akkus
Ferhan Candan
Abstract
C3
glomerulopati (C3G), kompleman aktivasyonunun anormal kontrolü ile oluşan bir grup
böbrek hastalığını kapsayan yakın zamanda tanımlanmış bir hastalıktır. C3G
glomerullerde immunoglobulin birikiminin trace ve yokluğu ile dominant olarak kompleman
C3 birikimi ile karakterizedir. C3G C3 glomerulonefrit (C3GN) ve Dens Deposit
Hastalığı (DDD) diye iki grup hastalıktan oluşmaktadır. DDD ve C3GN nadir
görülmektedir ve her iki hastalık hem çocuklarda hemde erişkinlerde görülmektedir.
C3G spesifik tedavisinde, plazmaferez, immünsüpresif tedavi ve kompleman inhibitörlerini
içermektedir.
References
- 1. Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, Teoh CW, Awan A, Waldron M, Cairns T, O'Kelly P, Dorman AM, Pickering MC, Conlon PJ, Cook HT. C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol 2014; 9: 46-53.
- 2. Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, Theis JD, Dogan A, Smith RJ. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int 2012; 82: 465-73.
- 3. Barbour TD, Pickering MC, Terence Cook H. Dense deposit disease and C3 glomerulopathy. Semin Nephrol 2013; 33: 493-507.
- 4. Viswanathan GK, Nada R, Kumar A, Ramachandran R, Rayat CS, Jha V, Sakhuja V, Joshi K. Clinico-pathologic spectrum of C3 glomerulopathy-an Indian experience. Diagn Pathol 2015; 10:6.
- 5. Pickering MC, D'agati VD, Nester CM, Smith RJ, Haas M, Appel GB, Doyle M et al. C3 glomerulopathy: consensus report. Kidney Int 2013; 84: 1079-89.
- 6. Walker PD. Dense doposit disease: New in sights. Curr Opin Nephrol Hypertens 2007; 16: 204-12.
- 7. Riedl M, Thorner P, Licht C. C3 Glomerulopathy. Pediatr Nephrol 2017; 32: 43-57.
- 8. Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, Smith RJ. Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int 2013; 83: 293-99.
- 9. Bridoux F, Desport E, Frémeaux-Bacchi V, Chong VF, Gombert JM, Lacombe C, Quellard N, Touchard G. Glomerulonephritiswith isolated C3 deposits and monoclonal gammopathy: a for tuitous association? Clin J Am Soc Nephrol 2011; 6: 2165-74.
- 10. Salvadori M, Rosso G. Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN. World J Nephrol 2016; 5: 308-20.
- 11. Khalighi MA, WangS, Henriksen KJ, Bock M, Keswani M, Meehan SM, Chang A. Revisiting post-infectious glomerulonephritis in the emergingera of C3 glomerulopathy. Clin Kidney J 2016; 9: 397-402.
- 12. Goodship THJ, Cook HT, Fakhouri F, Fer Fervenza FC Frémeaux-Bacchi V, Kavanagh D, Carla M. Nester CM, Noris M, Pickering MC, Rodríguez de Córdoba S.Roumenina LT, Sethi S, Smith RJH. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference. Kidney Int 2017; 91: 539-51.
- 13. Savige J, Amos L, Ierino F, Mack HG, Symons RC, Hughes P, Nicholls K, Colville D. Retinal disease in the C3 glomerulopathies and the risk of impaired vision. Ophthalmıc Genet 2016; 37: 369-76.
- 14. Cameron JS, Turner DR, Heaton J, Williams DG, Ogg CS, Chantler C, Hicks J. Idiopathic mesangiocapillary glomerulonephritis: Comparison of types I and II in children and adults and long-term prognosis. Am J Med. 1983; 74: 175-92.
- 15. Noris M, Remuzzi G. Overview of complement activation and regulation. Semin Nephrol 2013; 33: 479-92.
- 16. Zhang Y, Meyer NC, Wang K, Nishimura C, Frees K, Jones M, Katz LM, Sethi S, Smith RJ. Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am SocNephrol 2012; 7: 265-74.
- 17. Goodship TH, Pappworth IY, Toth T, Denton M, Houlberg K, McCormick F, Warland D, Moore I, Hunze EM, Staniforth SJ, Hayes C, Cavalcante DP, Kavanagh D, Strain L, Herbert AP, Schmidt CQ, Barlow PN, Harris CL, Marchbank KJ. Factor H autoantibodies in membranoproliferative glomerulonephritis. Mol Immunol 2012; 52: 200-6.
- 18. Strobel S, Zimmering M, Papp K, Prechl J, Józsi M. Anti-factor B autoantibody in dense deposit disease. Mol Immunol 2010; 47: 1476-83.
- 19. Servais A, Noël LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, Macher MA, Zuber J, Karras A, ProvotF, Moulin B, Grünfeld JP, Niaudet P, Lesavre P, Frémeaux-Bacchi V.Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 2012; 82: 454-64.
- 20. Gale DP, de Jorge EG, Cook HT, Martinez-Barricarte R, Hadjisavvas A, McLean AG, Pusey CD, Pierides A, Kyriacou K, Athanasiou Y, Voskarides K, Deltas C, Palmer A, Frémeaux-Bacchi V, de Cordoba SR, Maxwell PH, Pickering MC. Identification of a mutation in complement factor H-related protein 5 in patients of Cypriotorigin with glomerulonephritis. Lancet 2010; 376 (9743): 794-801.
- 21. Athanasiou Y, Voskarides K, Gale DP, Damianou L, Patsias C, Zavros M, Maxwell PH, Cook HT, Demosthenous P, Hadjisavvas A, Kyriacou K, Zouvani I, Pierides A, Deltas C. Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol 2011; 6: 1436-46.
- 22. Fakhouri F, Frémeaux-Bacchi V, Noël LH, Cook HT, Pickering MC. C3 glomerulopathy: A new classification. Nat Rev Nephrol 2010; 6: 494-9.
- 23. Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, Teoh CW, Awan A, Waldron M, Cairns T, O'Kelly P, Dorman AM, Pickering MC, Conlon PJ, Cook HT.C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol 2014; 9: 46-53.
- 24. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO Clinical Practice Guidline for Glomerulonephritis. General principles in the management of glomerular disease. Kidney Inter Suppl. 2012; 2: 156-62.
- 25. McCaughan JA, O'Rourke DM, Courtney AE. Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies. Am J Transplant 2012; 12: 1046-51.
- 26. Bomback AS, Appel GB. Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol 2012; 8: 634-42.
- 27. West CD, McAdams AJ, Witte DP. Acutenon-proliferative glomerulitis: a cause of renal failure unique to children. Pediatr Nephrol 2000; 14: 786-93.
- 28. Chen Q, Müller D, Rudolph B, Hartmann A, Kuwertz-Bröking E, Wu K, Kirschfink M, Skerka C, Zipfel PF. Combined c3b and factor B autoantibodies and MPGN type II. N Engl J Med 2011; 365: 2340-2.
- 29. Rabasco C, Cavero T, Román E, Rojas-Rivera J, Olea T, Espinosa M, Cabello V, Fernández-Juarez G, González F, Ávila A, Baltar JM, Díaz M, Alegre R, Elías S, Antón M, Frutos MA, Pobes A, Blasco M, Martín F, Bernis C, Macías M, Barroso S, de Lorenzo A, Ariceta G, López-Mendoza M, Rivas B, López-Revuelta K, Campistol JM, Mendizábal S, de Córdoba SR, Praga M. Spanish Group for the Study of Glomerular Diseases (GLOSEN). Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int 2015; 88: 1153-60.
- 30. De Vriese AS, Sethi S, Van Praet J, Nath KA, Fervenza FC. Kidney disease caused by dysregulation of the complement alternative pathway an etiology approach. J Am Soc Nephrol 2015; 26: 2917-29.
- 31. Licht C, Weyersberg A, Heinen S, Stapenhorst L, Devenge J, Beck B, Waldherr R, Kirschfink M, Zipfel PF, Hoppe B. Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15. Am J KidneyDis 2005; 45: 415-21.
- 32. Banks RA, May S, Wallington T. Acute renal failure in dense deposit disease: recovery after plasmapheresis. BrMed J (Clin Res Ed) 1982; 284: 1874-5.
- 33. Krmar RT, Holtbäck U, Linné T, Berg UB, Celsi G, Söderberg MP, Wernerson A, Szakos A, Larsson S, Skattum L, Bárány P. Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange. ClinNephrol 2011; 75 Suppl 1: 4-10.
- 34. Kurtz KA, Schlueter AJ. Management of membranoproliferative glomerulonephritis type II with plasmapheresis. J ClinApher 2002; 17: 135-7.
- 35. Häffner K, Michelfelder S, Pohl M. Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression. Pediatr Nephrol 2015; 30: 1951-9.
- 36. Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, Mele C, Bresin E, Cassis L, Gamba S, Porrati F, Bucchioni S, Monteferrante G, Fang CJ, Liszewski MK, Kavanagh D, Atkinson JP, Remuzzi G; Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood 2006; 1081: 267-79.
- 37. Tran H, Chaudhuri A, Concepcion W, Grimm PC. Use of eculizumab and plasma exchange in successful combined liver–kidney transplantation in a case of atypical HUS associated with complement factor H mutation. Pediatr Nephrol 2014; 29: 477-80.
- 38. Bomback AS, Smith RJ, Barile GR, Zhang Y, Heher EC, Herlitz L, Stokes MB, Markowitz GS, D'Agati VD, Canetta PA, Radhakrishnan J, Appel GB. Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 2012; 7: 748-56.
- 39. Oosterveld MJ, Garrelfs MR, Hoppe B, Florquin S, Roelofs JJ, van den Heuvel LP, Amann K, Davin JC, Bouts AH, Schriemer PJ, Groothoff JW. Eculizumab in Pediatric Dense Deposit Disease. Clin J Am SocNephrol 2015; 10: 1773-82.
- 40. Daina E, Noris M, Remuzzi G. Eculizumab in a patient with dense-deposit disease. N Engl J Med 2012; 366: 1161-3.
- 41. Inman M, Prater G, Fatima H, Wallace E. Eculizumab-induced reversal of dialysis-dependent kidney failure from C3 glomerulonephritis. Clin Kidney J 2015; 8: 445-8.
- 42. Payette A, Patey N, Dragon-Durey MA, Frémeaux-Bacchi V, Le Deist F, Lapeyraque AL. A case of C3 glomerulonephritis successfully treated with eculizumab. Pediatr Nephrol 2015; 30: 1033-7.
- 43. Le Quintrec M, Lionet A, Kandel C, Bourdon F, Gnemmi V, Colombat M, Goujon JM, Frémeaux-Bacchi V, Fakhouri F. Eculizumab for treatment of rapidly progressive C3 glomerulopathy. Am J Kidney Dis 2015; 65: 484-9.
- 44. Zhang Y, Nester CM, Holanda DG, Marsh HC, Hammond RA, Thomas LJ, Meyer NC, Hunsicker LG, Sethi S, Smith RJ. Soluble CR1 Therapy Improves Complement Regulation in C3 Glomerulopathy. J Am Soc Nephrol 2013; 24: 1820-9.
- 45. Nester CM, Smith RJ. Complement inhibition in c3 glomerulopathy. Semin Immunol 2016; 28: 241-9.
- 46. Zhang Y, Shao D, Ricklin D, Hilkin BM, Nester CM, Lambris JD, Smith RJ. Compstatin analog Cp40 inhibits complement dysregulation in vitro in C3 glomerulopathy. Immunobiology 2015; 220: 993-8.
- 47. D'souza Y, Short CD, McLeod D, Bonshek RE. Long-term follow-up of drusen-like lesions in patients with type II mesangiocapillary glomerulonephritis. Br J Ophthalmol 2008; 92: 950-3.
- 48. Khandhadia S, Hakobyan S, Heng LZ, Gibson J, Adams DH, Alexander GJ, Gibson JM, Martin KR, Menon G, Nash K, Sivaprasad S, Ennis S, Cree AJ, Morgan BP, Lotery AJ. Age-related macular degeneration and modification of systemic complement factor H production through liver transplantation. Ophthalmology 2013; 120: 1612-8.
- 49. Leys A, Michielsen B, Leys M, Vanrenterghem Y, Missotten L, Van Damme B. Subretinal neovascular membranes associated with chronic membranoproliferative glomerulonephritis type II. Graefes Arch Clin Exp Ophthalmol 1990; 2284 99-504.
- 50. Farah SE, Fazelat A, Frei G. Treatment of subretinal neovascular membrane in a patient with membranoproliferative glomerulonephritis type II. Ophthalmic Surgery, Lasers, and Imaging Retina 2009; 40: 416-8.
- 51. Lu DF, Moon M, Lanning LD, McCarthy AM, Smith RJ. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol 2012; 27: 773-81.
- 52. Zand L, Lorenz EC, Cosio FG, Fervenza FC, Nasr SH, Gandhi MJ, Smith RJ, Sethi S. Clinical findings, pathology, and outcomes of C3GN after kidney transplantation. J Am Soc Nephrol 2014; 25: 1110-7.
C3 glomerulopathy disease
Year 2018,
, 1 - 8, 20.03.2018
Can Huzmeli
,
Mehmet Hadi Akkus
Ferhan Candan
Abstract
C3
glomerulopathy is a recently defined disease that encompasses a group of kidney
diseases caused by abnormal control of complement activation. C3G is characterized
bydeposition of dominant complement C3 with absence/traces of immunoglobulins
in the glomeruli. C3G may be classified into
dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). DDD and
C3GN are rare forms of glomerulonephritis that affect both children and adults.
In treatment C3G include on specific treatment, plasma therapy, immunsupressive
and complement inhibition.
References
- 1. Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, Teoh CW, Awan A, Waldron M, Cairns T, O'Kelly P, Dorman AM, Pickering MC, Conlon PJ, Cook HT. C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol 2014; 9: 46-53.
- 2. Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, Theis JD, Dogan A, Smith RJ. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int 2012; 82: 465-73.
- 3. Barbour TD, Pickering MC, Terence Cook H. Dense deposit disease and C3 glomerulopathy. Semin Nephrol 2013; 33: 493-507.
- 4. Viswanathan GK, Nada R, Kumar A, Ramachandran R, Rayat CS, Jha V, Sakhuja V, Joshi K. Clinico-pathologic spectrum of C3 glomerulopathy-an Indian experience. Diagn Pathol 2015; 10:6.
- 5. Pickering MC, D'agati VD, Nester CM, Smith RJ, Haas M, Appel GB, Doyle M et al. C3 glomerulopathy: consensus report. Kidney Int 2013; 84: 1079-89.
- 6. Walker PD. Dense doposit disease: New in sights. Curr Opin Nephrol Hypertens 2007; 16: 204-12.
- 7. Riedl M, Thorner P, Licht C. C3 Glomerulopathy. Pediatr Nephrol 2017; 32: 43-57.
- 8. Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, Smith RJ. Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int 2013; 83: 293-99.
- 9. Bridoux F, Desport E, Frémeaux-Bacchi V, Chong VF, Gombert JM, Lacombe C, Quellard N, Touchard G. Glomerulonephritiswith isolated C3 deposits and monoclonal gammopathy: a for tuitous association? Clin J Am Soc Nephrol 2011; 6: 2165-74.
- 10. Salvadori M, Rosso G. Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN. World J Nephrol 2016; 5: 308-20.
- 11. Khalighi MA, WangS, Henriksen KJ, Bock M, Keswani M, Meehan SM, Chang A. Revisiting post-infectious glomerulonephritis in the emergingera of C3 glomerulopathy. Clin Kidney J 2016; 9: 397-402.
- 12. Goodship THJ, Cook HT, Fakhouri F, Fer Fervenza FC Frémeaux-Bacchi V, Kavanagh D, Carla M. Nester CM, Noris M, Pickering MC, Rodríguez de Córdoba S.Roumenina LT, Sethi S, Smith RJH. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference. Kidney Int 2017; 91: 539-51.
- 13. Savige J, Amos L, Ierino F, Mack HG, Symons RC, Hughes P, Nicholls K, Colville D. Retinal disease in the C3 glomerulopathies and the risk of impaired vision. Ophthalmıc Genet 2016; 37: 369-76.
- 14. Cameron JS, Turner DR, Heaton J, Williams DG, Ogg CS, Chantler C, Hicks J. Idiopathic mesangiocapillary glomerulonephritis: Comparison of types I and II in children and adults and long-term prognosis. Am J Med. 1983; 74: 175-92.
- 15. Noris M, Remuzzi G. Overview of complement activation and regulation. Semin Nephrol 2013; 33: 479-92.
- 16. Zhang Y, Meyer NC, Wang K, Nishimura C, Frees K, Jones M, Katz LM, Sethi S, Smith RJ. Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am SocNephrol 2012; 7: 265-74.
- 17. Goodship TH, Pappworth IY, Toth T, Denton M, Houlberg K, McCormick F, Warland D, Moore I, Hunze EM, Staniforth SJ, Hayes C, Cavalcante DP, Kavanagh D, Strain L, Herbert AP, Schmidt CQ, Barlow PN, Harris CL, Marchbank KJ. Factor H autoantibodies in membranoproliferative glomerulonephritis. Mol Immunol 2012; 52: 200-6.
- 18. Strobel S, Zimmering M, Papp K, Prechl J, Józsi M. Anti-factor B autoantibody in dense deposit disease. Mol Immunol 2010; 47: 1476-83.
- 19. Servais A, Noël LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, Macher MA, Zuber J, Karras A, ProvotF, Moulin B, Grünfeld JP, Niaudet P, Lesavre P, Frémeaux-Bacchi V.Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int 2012; 82: 454-64.
- 20. Gale DP, de Jorge EG, Cook HT, Martinez-Barricarte R, Hadjisavvas A, McLean AG, Pusey CD, Pierides A, Kyriacou K, Athanasiou Y, Voskarides K, Deltas C, Palmer A, Frémeaux-Bacchi V, de Cordoba SR, Maxwell PH, Pickering MC. Identification of a mutation in complement factor H-related protein 5 in patients of Cypriotorigin with glomerulonephritis. Lancet 2010; 376 (9743): 794-801.
- 21. Athanasiou Y, Voskarides K, Gale DP, Damianou L, Patsias C, Zavros M, Maxwell PH, Cook HT, Demosthenous P, Hadjisavvas A, Kyriacou K, Zouvani I, Pierides A, Deltas C. Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol 2011; 6: 1436-46.
- 22. Fakhouri F, Frémeaux-Bacchi V, Noël LH, Cook HT, Pickering MC. C3 glomerulopathy: A new classification. Nat Rev Nephrol 2010; 6: 494-9.
- 23. Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, Teoh CW, Awan A, Waldron M, Cairns T, O'Kelly P, Dorman AM, Pickering MC, Conlon PJ, Cook HT.C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol 2014; 9: 46-53.
- 24. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO Clinical Practice Guidline for Glomerulonephritis. General principles in the management of glomerular disease. Kidney Inter Suppl. 2012; 2: 156-62.
- 25. McCaughan JA, O'Rourke DM, Courtney AE. Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies. Am J Transplant 2012; 12: 1046-51.
- 26. Bomback AS, Appel GB. Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol 2012; 8: 634-42.
- 27. West CD, McAdams AJ, Witte DP. Acutenon-proliferative glomerulitis: a cause of renal failure unique to children. Pediatr Nephrol 2000; 14: 786-93.
- 28. Chen Q, Müller D, Rudolph B, Hartmann A, Kuwertz-Bröking E, Wu K, Kirschfink M, Skerka C, Zipfel PF. Combined c3b and factor B autoantibodies and MPGN type II. N Engl J Med 2011; 365: 2340-2.
- 29. Rabasco C, Cavero T, Román E, Rojas-Rivera J, Olea T, Espinosa M, Cabello V, Fernández-Juarez G, González F, Ávila A, Baltar JM, Díaz M, Alegre R, Elías S, Antón M, Frutos MA, Pobes A, Blasco M, Martín F, Bernis C, Macías M, Barroso S, de Lorenzo A, Ariceta G, López-Mendoza M, Rivas B, López-Revuelta K, Campistol JM, Mendizábal S, de Córdoba SR, Praga M. Spanish Group for the Study of Glomerular Diseases (GLOSEN). Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int 2015; 88: 1153-60.
- 30. De Vriese AS, Sethi S, Van Praet J, Nath KA, Fervenza FC. Kidney disease caused by dysregulation of the complement alternative pathway an etiology approach. J Am Soc Nephrol 2015; 26: 2917-29.
- 31. Licht C, Weyersberg A, Heinen S, Stapenhorst L, Devenge J, Beck B, Waldherr R, Kirschfink M, Zipfel PF, Hoppe B. Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15. Am J KidneyDis 2005; 45: 415-21.
- 32. Banks RA, May S, Wallington T. Acute renal failure in dense deposit disease: recovery after plasmapheresis. BrMed J (Clin Res Ed) 1982; 284: 1874-5.
- 33. Krmar RT, Holtbäck U, Linné T, Berg UB, Celsi G, Söderberg MP, Wernerson A, Szakos A, Larsson S, Skattum L, Bárány P. Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange. ClinNephrol 2011; 75 Suppl 1: 4-10.
- 34. Kurtz KA, Schlueter AJ. Management of membranoproliferative glomerulonephritis type II with plasmapheresis. J ClinApher 2002; 17: 135-7.
- 35. Häffner K, Michelfelder S, Pohl M. Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression. Pediatr Nephrol 2015; 30: 1951-9.
- 36. Caprioli J, Noris M, Brioschi S, Pianetti G, Castelletti F, Bettinaglio P, Mele C, Bresin E, Cassis L, Gamba S, Porrati F, Bucchioni S, Monteferrante G, Fang CJ, Liszewski MK, Kavanagh D, Atkinson JP, Remuzzi G; Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood 2006; 1081: 267-79.
- 37. Tran H, Chaudhuri A, Concepcion W, Grimm PC. Use of eculizumab and plasma exchange in successful combined liver–kidney transplantation in a case of atypical HUS associated with complement factor H mutation. Pediatr Nephrol 2014; 29: 477-80.
- 38. Bomback AS, Smith RJ, Barile GR, Zhang Y, Heher EC, Herlitz L, Stokes MB, Markowitz GS, D'Agati VD, Canetta PA, Radhakrishnan J, Appel GB. Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 2012; 7: 748-56.
- 39. Oosterveld MJ, Garrelfs MR, Hoppe B, Florquin S, Roelofs JJ, van den Heuvel LP, Amann K, Davin JC, Bouts AH, Schriemer PJ, Groothoff JW. Eculizumab in Pediatric Dense Deposit Disease. Clin J Am SocNephrol 2015; 10: 1773-82.
- 40. Daina E, Noris M, Remuzzi G. Eculizumab in a patient with dense-deposit disease. N Engl J Med 2012; 366: 1161-3.
- 41. Inman M, Prater G, Fatima H, Wallace E. Eculizumab-induced reversal of dialysis-dependent kidney failure from C3 glomerulonephritis. Clin Kidney J 2015; 8: 445-8.
- 42. Payette A, Patey N, Dragon-Durey MA, Frémeaux-Bacchi V, Le Deist F, Lapeyraque AL. A case of C3 glomerulonephritis successfully treated with eculizumab. Pediatr Nephrol 2015; 30: 1033-7.
- 43. Le Quintrec M, Lionet A, Kandel C, Bourdon F, Gnemmi V, Colombat M, Goujon JM, Frémeaux-Bacchi V, Fakhouri F. Eculizumab for treatment of rapidly progressive C3 glomerulopathy. Am J Kidney Dis 2015; 65: 484-9.
- 44. Zhang Y, Nester CM, Holanda DG, Marsh HC, Hammond RA, Thomas LJ, Meyer NC, Hunsicker LG, Sethi S, Smith RJ. Soluble CR1 Therapy Improves Complement Regulation in C3 Glomerulopathy. J Am Soc Nephrol 2013; 24: 1820-9.
- 45. Nester CM, Smith RJ. Complement inhibition in c3 glomerulopathy. Semin Immunol 2016; 28: 241-9.
- 46. Zhang Y, Shao D, Ricklin D, Hilkin BM, Nester CM, Lambris JD, Smith RJ. Compstatin analog Cp40 inhibits complement dysregulation in vitro in C3 glomerulopathy. Immunobiology 2015; 220: 993-8.
- 47. D'souza Y, Short CD, McLeod D, Bonshek RE. Long-term follow-up of drusen-like lesions in patients with type II mesangiocapillary glomerulonephritis. Br J Ophthalmol 2008; 92: 950-3.
- 48. Khandhadia S, Hakobyan S, Heng LZ, Gibson J, Adams DH, Alexander GJ, Gibson JM, Martin KR, Menon G, Nash K, Sivaprasad S, Ennis S, Cree AJ, Morgan BP, Lotery AJ. Age-related macular degeneration and modification of systemic complement factor H production through liver transplantation. Ophthalmology 2013; 120: 1612-8.
- 49. Leys A, Michielsen B, Leys M, Vanrenterghem Y, Missotten L, Van Damme B. Subretinal neovascular membranes associated with chronic membranoproliferative glomerulonephritis type II. Graefes Arch Clin Exp Ophthalmol 1990; 2284 99-504.
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