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Fibrillary Glomerulonephritis

  • Charles E. Alpers
Living reference work entry

Abstract

Fibrillary glomerulonephritis is a progressive glomerulopathy characterized by the deposition of abnormal fibrils having an ultrastructural appearance similar to amyloid fibrils that are likely composed of abnormal immunoglobulins. It is a morphologically defined entity that is somewhat clinically heterogeneous. The precise etiology for this process is unknown. A kidney biopsy and, in particular electron microscopy, is required for establishing the diagnosis. There is a strong propensity to end-stage renal disease. Randomized controlled clinical trials of therapies for this disease remain to be reported.

Keywords

Fibrillary glomerulonephritis Fibrils Mesangium Basement membranes Electron microscopy 

References

  1. Alpers CE, Kowalewska J (2008) Fibrillary glomerulonephritis and immunotactoid glomerulopathy. J Am Soc Nephrol 19:34–37CrossRefPubMedGoogle Scholar
  2. Hemminger J, Nadasdy G, Satoskar A, Brodsky SV, Nadasdy T (2016) IgG subclass staining in routine renal biopsy material. Am J Surg Pathol 40:617–626CrossRefPubMedGoogle Scholar
  3. Hogan J, Restivo M, Canetta PA, Herlitz LC, Radhakrishnan J, Appel GB, Bomback AS (2014) Rituximab treatment for fibrillary glomerulonephritis. Nephrol Dial Transplant 29:1925–1931CrossRefPubMedGoogle Scholar
  4. Javaugue V, Karras A, Glowacki F, McGregor B, Lacombe C, Goujon JM, Ragot S, Aucouturier P, Touchard G, Bridoux F (2013) Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients. Am J Kidney Dis 62:679–690CrossRefPubMedGoogle Scholar
  5. Mallett A, Tang W, Hart G, McDonald SP, Hawley CM, Badve SV, Boudville N, Brown FG, Campbell SB, Clayton PA, Johnson DW (2015) End-stage kidney disease due to fibrillary glomerulonephritis and immunotactoid glomerulopathy – outcomes in 66 consecutive ANZDATA registry C. Am J Nephrol 42:177–184CrossRefPubMedGoogle Scholar
  6. Nasr SH, Valeri AM, Cornell LD, Fidler ME, Sethi S, Leung N, Fervenza FC (2011) Fibrillary glomerulonephritis: a report of 66 cases from a single institution. Clin J Am Soc Nephrol 6:775–784CrossRefPubMedPubMedCentralGoogle Scholar
  7. Sethi S, Theis JD, Vrana JA, Fervenza FC, Sethi A, Qian Q, Quint P, Leung N, Dogan A, Nasr SH (2013) Laser microdissection and proteomic analysis of amyloidosis, cryoglobulinemic GN, fibrillary GN, and immunotactoid glomerulopathy. Clin J Am Soc Nephrol 8:915–921CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of PathologyUniversity of Washington Medical CenterSeattleUSA

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