Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Glomerular diseases due to nonamyloid fibrillar deposits

Fernando C Fervenza, MD, PhD
Sanjeev Sethi, MD, PhD
Section Editors
Richard J Glassock, MD, MACP
Brad H Rovin, MD
Deputy Editor
Albert Q Lam, MD


Glomerular disease may be associated with the appearance of fibrillar deposits in the mesangium or glomerular basement membrane. The fibrils are Congo red-positive in amyloidosis and Congo red-negative in other forms. The most common forms of Congo red-negative (nonamyloid) fibrillar glomerular deposition diseases are fibrillary glomerulonephritis and immunotactoid glomerulopathy.

Fibrillary glomerulonephritis [1-6] and immunotactoid glomerulopathy [3,5-9] are uncommon causes of glomerular disease. Both disorders probably result from deposits derived from immunoglobulins [9]. The pathognomonic histologic findings are seen on electron microscopy. By comparison, other forms of nonamyloid fibrillary deposition disease have been described in which the deposits are composed of fibronectin (fibronectin glomerulopathy) or atypical type III collagen fibrils (collagenofibrotic glomerulopathy). (See 'Pathology and pathogenesis' below and 'Other fibrillary disorders' below.)

Glomerular disease associated with amyloid deposits is discussed separately. (See "Renal amyloidosis".)


Fibrillary glomerulonephritis and immunotactoid glomerulopathy are uncommon disorders, being present in 0.5 to 1 percent of native renal biopsies [3,6]. Most investigators feel that, based upon histologic features and clinical associations, fibrillary glomerulonephritis and immunotactoid glomerulopathy are separate disorders, with fibrillary glomerulonephritis accounting for approximately 85 to 90 percent of cases [3,5,8]. However, some believe that they are pathogenetically related, with no compelling histologic or clinical reasons to separate the two. (See 'One or two disorders?' below.)

Pathology and pathogenesis

Fibrillary glomerulonephritis — The diagnosis of fibrillary glomerulonephritis is established by renal biopsy, with the pathognomonic changes seen on electron microscopy.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Dec 01, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Alpers CE, Rennke HG, Hopper J Jr, Biava CG. Fibrillary glomerulonephritis: an entity with unusual immunofluorescence features. Kidney Int 1987; 31:781.
  2. Iskandar SS, Falk RJ, Jennette JC. Clinical and pathologic features of fibrillary glomerulonephritis. Kidney Int 1992; 42:1401.
  3. Rosenstock JL, Markowitz GS, Valeri AM, et al. Fibrillary and immunotactoid glomerulonephritis: Distinct entities with different clinical and pathologic features. Kidney Int 2003; 63:1450.
  4. Nasr SH, Valeri AM, Cornell LD, et al. Fibrillary glomerulonephritis: a report of 66 cases from a single institution. Clin J Am Soc Nephrol 2011; 6:775.
  5. Pronovost PH, Brady HR, Gunning ME, et al. Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy. Nephrol Dial Transplant 1996; 11:837.
  6. Fogo A, Qureshi N, Horn RG. Morphologic and clinical features of fibrillary glomerulonephritis versus immunotactoid glomerulopathy. Am J Kidney Dis 1993; 22:367.
  7. Korbet SM, Schwartz MM, Lewis EJ. Immunotactoid glomerulopathy. Am J Kidney Dis 1991; 17:247.
  8. Alpers CE. Immunotactoid (microtubular) glomerulopathy: an entity distinct from fibrillary glomerulonephritis? Am J Kidney Dis 1992; 19:185.
  9. Schwartz MM, Korbet SM, Lewis EJ. Immunotactoid glomerulopathy. J Am Soc Nephrol 2002; 13:1390.
  10. Yang GC, Nieto R, Stachura I, Gallo GR. Ultrastructural immunohistochemical localization of polyclonal IgG, C3, and amyloid P component on the congo red-negative amyloid-like fibrils of fibrillary glomerulopathy. Am J Pathol 1992; 141:409.
  11. Bridoux F, Hugue V, Coldefy O, et al. Fibrillary glomerulonephritis and immunotactoid (microtubular) glomerulopathy are associated with distinct immunologic features. Kidney Int 2002; 62:1764.
  12. Thomas JA, Vasin D, Lin M, et al. A case of mistaken identity: fibrillary glomerulonephritis masquerading as crescentic anti-glomerular basement membrane disease. Clin Nephrol 2016; 85:114.
  13. Dasari S, Alexander MP, Vrana JA, et al. DnaJ Heat Shock Protein Family B Member 9 Is a Novel Biomarker for Fibrillary GN. J Am Soc Nephrol 2017.
  14. Andeen NK, Yang HY, Dai DF, et al. DnaJ Homolog Subfamily B Member 9 Is a Putative Autoantigen in Fibrillary GN. J Am Soc Nephrol 2017.
  15. Nasr SH, Vrana JA, Dasari S, et al. DNAJB9 Is a Specific Immunohistochemical Marker for Fibrillary Glomerulonephritis. Kidney Int Rep 2017.
  16. Nickeleit V, Moll S, Schmid M, Mihatsch MJ. Nephrotic syndrome in an adult: the ongoing saga of fibrils versus microtubules. Am J Kidney Dis 1999; 34:1146.
  17. Korbet SM, Rosenberg BF, Schwartz MM, Lewis EJ. Course of renal transplantation in immunotactoid glomerulopathy. Am J Med 1990; 89:91.
  18. Czarnecki PG, Lager DJ, Leung N, et al. Long-term outcome of kidney transplantation in patients with fibrillary glomerulonephritis or monoclonal gammopathy with fibrillary deposits. Kidney Int 2009; 75:420.
  19. Masson RG, Rennke HG, Gottlieb MN. Pulmonary hemorrhage in a patient with fibrillary glomerulonephritis. N Engl J Med 1992; 326:36.
  20. Wallner M, Prischl FC, Höbling W, et al. Immunotactoid glomerulopathy with extrarenal deposits in the bone, and chronic cholestatic liver disease. Nephrol Dial Transplant 1996; 11:1619.
  21. Moulin B, Ronco PM, Mougenot B, et al. Glomerulonephritis in chronic lymphocytic leukemia and related B-cell lymphomas. Kidney Int 1992; 42:127.
  22. Brady HR. Fibrillary glomerulopathy. Kidney Int 1998; 53:1421.
  23. Markowitz GS, Cheng JT, Colvin RB, et al. Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy. J Am Soc Nephrol 1998; 9:2244.
  24. Cheungpasitporn W, Zacharek CC, Fervenza FC, et al. Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. Clin Kidney J 2016; 9:97.
  25. Bjørneklett R, Vikse BE, Svarstad E, et al. Long-term risk of cancer in membranous nephropathy patients. Am J Kidney Dis 2007; 50:396.
  26. Javaugue V, Karras A, Glowacki F, et al. Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients. Am J Kidney Dis 2013; 62:679.
  27. Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol 2011; 31:341.
  28. D'Agati V, Sacchi G, Truong L, et al. Fibrillary glomerulopathy: Defining the disease spectrum [abstract]. J Am Soc Nephrol 1991; 2:591.
  29. Collins M, Navaneethan SD, Chung M, et al. Rituximab treatment of fibrillary glomerulonephritis. Am J Kidney Dis 2008; 52:1158.
  30. Bhat P, Weiss S, Appel GB, Radhakrishnan J. Rituximab treatment of dysproteinemias affecting the kidney: a review of three cases. Am J Kidney Dis 2007; 50:641.
  31. Hogan J, Restivo M, Canetta PA, et al. Rituximab treatment for fibrillary glomerulonephritis. Nephrol Dial Transplant 2014; 29:1925.
  32. Carles X, Rostaing L, Modesto A, et al. Successful treatment of recurrence of immunotactoid glomerulopathy in a kidney allograft recipient. Nephrol Dial Transplant 2000; 15:897.
  33. Mahajan S, Kalra V, Dinda AK, et al. Fibrillary glomerulonephritis presenting as rapidly progressive renal failure in a young female: a case report. Int Urol Nephrol 2005; 37:561.
  34. Sharma P, Kuperman M, Racusen L, Geetha D. Fibrillary glomerulonephritis presenting as rapidly progressive glomerulonephritis. Am J Kidney Dis 2012; 60:157.
  35. Vilayur E, Trevillian P, Walsh M. Monoclonal gammopathy and glomerulopathy associated with chronic lymphocytic leukemia. Nat Clin Pract Nephrol 2009; 5:54.
  36. Marin JM, Agusti A, Villar I, et al. Association between treated and untreated obstructive sleep apnea and risk of hypertension. JAMA 2012; 307:2169.
  37. Mallett A, Tang W, Hart G, et al. End-Stage Kidney Disease Due to Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy - Outcomes in 66 Consecutive ANZDATA Registry Cases. Am J Nephrol 2015; 42:177.
  38. Sathyan S, Khan FN, Ranga KV. A case of recurrent immunotactoid glomerulopathy in an allograft treated with rituximab. Transplant Proc 2009; 41:3953.
  39. Otsuka Y, Takeda A, Horike K, et al. A recurrent fibronectin glomerulopathy in a renal transplant patient: a case report. Clin Transplant 2012; 26 Suppl 24:58.
  40. Alchi B, Nishi S, Narita I, Gejyo F. Collagenofibrotic glomerulopathy: clinicopathologic overview of a rare glomerular disease. Am J Kidney Dis 2007; 49:499.
  41. Chen N, Pan X, Xu Y, et al. Two brothers in one Chinese family with collagen type III glomerulopathy. Am J Kidney Dis 2007; 50:1037.
  42. Roberts IS, Gleadle JM. Familial nephropathy and multiple exostoses with exostosin-1 (EXT1) gene mutation. J Am Soc Nephrol 2008; 19:450.
  43. Sethi S, Theis JD, Leung N, et al. Mass spectrometry-based proteomic diagnosis of renal immunoglobulin heavy chain amyloidosis. Clin J Am Soc Nephrol 2010; 5:2180.