Kidney nontumor / medical renal

Monoclonal gammopathy of renal significance (MGRS) / paraprotein-related kidney disease

Immunotactoid glomerulopathy

Last author update: 2 July 2012
Last staff update: 20 September 2023

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PubMed Search: Immunotactoid glomerulopathy

Nikhil Sangle, M.D.
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Cite this page: Sangle N. Immunotactoid glomerulopathy. website. Accessed September 30th, 2023.
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
Case reports
Microscopic (histologic) description
  • Mesangial widening and occasional hypercellularity, capillary wall thickening; 25% have crescents
Microscopic (histologic) images

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Various images including EM

Immunofluorescence description
  • Deposition of usually monoclonal IgG and C3 in glomeruli
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

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Figures e - h

With fibrillary glomerulonephritis

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