Severe active C3 glomerulonephritis triggered by immune complexes and inactivated after eculizumab therapy

Diagnostic Pathology

Volumn 11, Issue 1, 2016, Pages

  Kersnik Levart, Tanja ^a   Ferluga, Dušan ^b   Vizjak, Alenka ^b   Mraz, Jerica ^b   Kojc, Nika ^b  

^a UNIVERSITY MEDICAL CENTRE LJUBLJANA   (Slovenia)

^b UNIVERSITY OF LJUBLJANA   (Slovenia)

Author keywords

C3 glomerulonephritis; Complement alternative pathway dysregulation; Eculizumab; Membranoproliferative glomerulonephritis

Indexed keywords

BIOLOGICAL MARKER; C3 PROTEIN, HUMAN; COMPLEMENT COMPONENT C3; COMPLEMENT INHIBITOR; ECULIZUMAB; MONOCLONAL ANTIBODY;

ADOLESCENT; BIOPSY; CASE REPORT; COMPLEMENT ALTERNATIVE PATHWAY; DRUG EFFECTS; ELECTRON MICROSCOPY; FLUORESCENT ANTIBODY TECHNIQUE; GLOMERULONEPHRITIS, MEMBRANOPROLIFERATIVE; GLOMERULUS; HUMAN; IMMUNOLOGY; MALE; PATHOLOGY; REMISSION; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME; ULTRASTRUCTURE;

ADOLESCENT; ANTIBODIES, MONOCLONAL, HUMANIZED; BIOMARKERS; BIOPSY; COMPLEMENT C3; COMPLEMENT INACTIVATING AGENTS; COMPLEMENT PATHWAY, ALTERNATIVE; FLUORESCENT ANTIBODY TECHNIQUE; GLOMERULONEPHRITIS, MEMBRANOPROLIFERATIVE; HUMANS; KIDNEY GLOMERULUS; MALE; MICROSCOPY, ELECTRON; REMISSION INDUCTION; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME;

EID: 84990841054     PISSN: None     EISSN: 17461596     Source Type: Journal    
DOI: 10.1186/s13000-016-0547-6     Document Type: Article

References (31)

1. Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol. 2011;31:341-8.
2. Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis - a new look at an old entity. N Engl J Med. 2012;366:1119-31.
3. Sethi S, Nester CM, Smith RJ. Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion. Kidney Int. 2012;5:434-41.
4. Pickering MC, D'Agati VD, Nester CM, Smith JR, Haas M, Appel BG, et al. C3 glomerulopathy: consensus report. Kidney Int. 2013;84:1079-89.
5. Cook HT. Complement and kidney disease. Curr Opin Nephrol Hypertens. 2013;22:295-301.
6. Medjeral-Thomas NR, O'Shaughnessy MM, O'Regan JA, Traynor C, Flanagan M, Wong L, et al. C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol. 2014;9:46-53.
7. Noris M, Remuzzi G. Glomerular diseases dependent on complement activation, including atypical hemolytic uremic syndrome, membranoproliferative glomerulonephritis, and C3 glomerulopathy: core curriculum 2015. Am J Kidney Dis. 2015;66:359-75.
8. Hou J, Markowitz GS, Bomback AS, Appel GB, Herlitz LC, Barry Stokes M, et al. Toward a working definition of C3 glomerulopathy by immunofluorescence. Kidney Int. 2014;85:450-6.
9. Pickering M, Cook HT. Complement and glomerular disease: new insights. Curr Opin Nephrol Hypertens. 2011;20:271-7.
10. Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, et al. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int. 2012;82:465-73.
11. Xiao X, Pickering MC, Smith RJH. C3 glomerulopathy: the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis. Semin Thromb Hemost. 2014;40:465-71.
12. Abrera-Abeleda MA, Nishimura C, Smith JL, Sethi S, McRae JL, Murphy BF, et al. Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease). J Med Genet. 2006;43:582-9.
13. Neumann HP, Salzmann M, Bohnert-Iwan B, Mannuelian T, Skerka C, Lenk D, et al. Haemolytic uraemic syndrome in mutations of the factor H gene: a registry based study of German speaking countries. J Med Genet. 2003;40:676-81.
14. Caprioli J, Castekketti F, Bucchioni S, Bettinaglio P, Bresin E, Pianetti G, et al. Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257 T, the A2089G and the G2881T polymorphisms are strongly associated with the disease. Hum Mol Genet. 2003;12:3385-95.
15. Radhakrishnan S, Lunn A, Kirschfink M, Thormer P, Hebert D, Langlois V, et al. Eculizumab and refractory membranoproliferative glomerulonephritis. N Engl J Med. 2012;366:1165-6.
16. Bomback AS, Smith RJ, Barile GR, Zhang Y, Heher CE, Herlitz L, et al. Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol. 2012;7:748-56.
17. Zuber J, Fakhouri F, Roumenina LT, Loirat C. Frémeaux-Bacchi on behalf of the French Study Group for aHUS/C3G. Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nature Rev Nephrol. 2012;8:643-57.
18. Herlitz LC, Bomback AS, Markowitz GS, Stokes MB, Smith RN, Colvin RB, et al. Pathology after eculizumab in dense deposit disease and C3 GN. J Am Soc Nephrol. 2012;23:1229-37.
19. Gurkan S, Fyfe B, Weiss L, Xiao X, Zhang Y, Smith RJ. Eculizumab and recurrent C3 glomerulonephritis. Pediatr Nephrol. 2013;28:1975-81.
20. Kerns E, Rozansky D, Troxell ML. Evolution of immunoglobulin deposition in C3-dominant membranoproliferative glomerulopathy. Pediatr Nephrol. 2013;28:2227-31.
21. Vivarelli M, Emma F. Treatment of C3 glomerulopathy with complement blockers. Semin Thromb Hemost. 2014;40:472-7.
22. Bomback AS. Eculizumab in the treatment of membranoproliferative glomerulonephritis. Nephron Clin Pract. 2014;3-4:270-6.
23. Payatte A, Patey N, Dragon-Durey MA, Fremeaux-Bacchi V, Le Deist F, Lapeyraque AL. A case of C3 glomerulonephritis successfully treated with eculizumab. Pediatr Nephrol. 2015;6:1033-7.
24. Le Quintrec M, Lionet A, Kandel C, Bourdon F, Gnemmi V, Colombat M, et al. Eculizumab for treatment of rapidly progressive C3 glomerulopathy. Am J Kidney Dis. 2015;3:484-9.
25. Inman M, Prater G, Fatima H, Wallace E. Eculizumab-induced reversal of dialysis-dependent kidney failure from C3 glomerulonephritis. Clin Kidney J. 2015;4:445-8.
26. Servais A, Noel LH, Roumenina LT, Le Quintec M, Ngo S, Dragon-Durey MA, et al. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int. 2012;4:454-64.
27. Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, et al. Atypical post-infectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int. 2013;83:293-9.
28. Sandhu G, Bansal A, Ranade A, Jones J, Cortel S, Markowitz G. C3 glomerulopathy masquerading as acute postinfectious glomerulonephritis. Am J Kidney Dis. 2012;60:1039-43.
29. Nester C, Brophy PD. Eculizumab in the treatment of atypical haemolytic uraemic syndrome and other complement-mediated renal diseases. Curr Opin Pediatr. 2013;25:225-31.
30. Rodriguez-Osorio L, Ortiz A. Timing of eculizumab therapy fpr C3 glomerulonephritis. Clin Kidney J. 2015;4:449-52.
31. Hillmen P, Elebute M, Kelly R, Urbano-Ispizua A, Hill A, Rother RP, et al. Long-term effect of the complement inhibitor eculizumab on kidney function in patients with paroxysmal nocturnal hemoglobinuria. Am J Hematol. 2010;85:553-9.