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Kidney non-tumor

Primary glomerular diseases

Collapsing glomerulopathy


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 4 September 2012, last major update September 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Resembles special form of focal and segmental glomerulosclerosis in IV drug abuse and AIDS (rapid progression to renal failure with severe proteinuria, poor response to treatment and similar microscopic changes), but HIV negative
● Usually black men; associated with genetic variants in the nonmuscle myosin heavy chain 9 gene (MYH9) (Semin Nephrol 2010;30:111)
● May be due to altered hemodynamics
● Renal allografts: important cause of graft dysfunction that may lead to allograft failure (Indian J Nephrol 2011;21:10); similar histologic changes, but process may have different origin (Hum Pathol 2002;33:437)
● May be associated with various disorders, which may have podocyte injury in common: mixed connective tissue disease (Clin Nephrol 2011;75 Suppl 1:32), natural killer cell leukeima (Am J Kidney Dis 2011;58:855), sickle cell disease (J Med Case Reports 2011;5:71), sirolimus treatment (Ann Transplant 2011;16:113), systemic lupus erythematosus or lupus-like disease (Clin J Am Soc Nephrol 2012 Mar 29 [Epub ahead of print]), tuberculosis (Ren Fail 2010;32:143)

Case reports
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● 58 year old man effectively treated with lisinopril and deflazacort (Clin Exp Nephrol 2010;14:385)

Micro description
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● Accentuation of lobules due to widespread collapse of glomerular capillary loops with localized hyperplasia and hypertrophy of epithelial cells overlying sclerotic segment
● Cells are vacuolated and swollen, contain abundant resorption droplets
● Severe tubulointerstitial injury with degenerative changes, dilated tubules, tubular casts and lymphocytic infiltrate

Micro images
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Collapsed glomerulus

       
With other disorders (left: Adult-onset Still's disease, middle: systemic lupus erythematosus, right: sickle cell disease)

   
Series of images

Immunofluorescence
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● Segmental IgM and C3

Electron microscopy description
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● Wrinkling but little/no basement membrane thickening; marked podocyte hypertrophy; no electron-dense deposits of tubuloreticular inclusions (compared to HIV associated nephropathy)

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

Ref Updated: 4/20/12


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