Kidney nontumor
Primary glomerular diseases
Immunotactoid glomerulopathy

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 21 September 2017, last major update July 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Immunotactoid glomerulopathy

Cite this page: Sangle, N. Immunotactoid glomerulopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyimmunotactoid.html. Accessed November 21st, 2017.
Definition / general
  • Rare ( < 1% of renal biopsies) disorder with extracellular glomerular deposition of nonamyloid fibrils
  • Patients have monoclonal immunoglobulin deposition in glomeruli and may have circulating paraproteins
  • More common in whites and females
  • Related to fibrillary glomerulonephritis but different fibril size and arrangement
  • May overlap with hepatitis C virus induced cryoglobulinemic glomerulonephritis
Clinical features
  • Presents with nephrotic syndrome
  • Patients with circulating or urinary paraproteins are more likely to have lymphoproliferative disorders
  • Poor long term survival
  • Diagnosis based on EM findings and exclusion of other possible causes of fibrillary deposits, such as amyloidosis, cryoglobulinemia, systemic lupus erythematosus or paraproteinemia
Treatment
Case reports
Microscopic (histologic) description
  • Mesangial widening and occasional hypercellularity, capillary wall thickening; 25% have crescents
Microscopic (histologic) images

Images hosted on other servers:

Various images including EM

Immunofluorescence
  • Variable IgG, C3; occasional IgM, IgA
Negative stains
Electron microscopy description
  • Extracellular, nonamyloid deposits 30 - 50 nm wide, focally arranged in parallel arrays and with a visible lumen (microtubules) usually within mesangium but also involving basement membrane
  • In comparison, fibrillary glomerulonephritis has smaller fibrils, 10 - 30 nm diameter with only focal parallel arrangement
Electron microscopy images

Images hosted on other servers:

Various images

Figures e - h

With fibrillary glomerulonephritis