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Kidney non-tumor

Primary glomerular diseases

Immunotactoid glomerulopathy


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 2 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● 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
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● 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

Treatments
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● Hypertensive control, possibly steroids (Clin Exp Nephrol 2009;13:378), possibly Rituximab (Transplant Proc 2009;41:3953), kidney transplant

Case reports
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● 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)

Micro description
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● Mesangial widening and occasional hypercellularity, capillary wall thickening; 25% have crescents

Micro images
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Various images including EM

Immunofluorescence
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● Variable IgG, C3; occasional IgM, IgA

Negative stains
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● Congo red, thioflavin T

Electron microscopy description
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● Extracellular, non-amyloid 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, 1030 nm diameter, with only focal parallel arrangement

Electron microscopy images
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Various images


Figures e-h

       
With fibrillary glomerulonephritis

End of Kidney non-tumor > Primary glomerular diseases > Immunotactoid glomerulopathy

Ref Updated: 5/25/12


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