Kidney nontumor / medical renal
Monoclonal gammopathy of renal significance (MGRS) / paraprotein-related kidney disease
Immunotactoid glomerulopathy
Author: Nikhil Sangle, M.D.
Last author update: 2 July 2012
Last staff update: 20 September 2023
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Immunotactoid glomerulopathy
Table of Contents
Definition / general | Clinical features | Treatment | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Immunofluorescence description | Negative stains | Electron microscopy description | Electron microscopy imagesCite this page: Sangle N. Immunotactoid glomerulopathy. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneyimmunotactoid.html. Accessed December 18th, 2024.
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
- Hypertensive control, possibly steroids, possibly rituximab, kidney transplant (Clin Exp Nephrol 2009;13:378, Transplant Proc 2009;41:3953)
Case reports
- 43 year old woman with spontaneous remission (Neth J Med 2011;69:341)
- 59 year old woman with proteinuria, immunotactoid glomerulopathy, heavy chain disease and follicular lymphoma (Arch Pathol Lab Med 2004;128:689)
- 69 year old man with lobular glomerulonephritis (Clin Nephrol 2005;63:368)
- Progression to end stage renal disease within 1 week of initial presentation (ScientificWorldJournal 2009;9:1348)
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 description
- Deposition of usually monoclonal IgG and C3 in glomeruli
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:
Figures e - h
With fibrillary glomerulonephritis
