CASE OF THE FORTNIGHT

 
 

Case 1
May 08

Collapsing Glomerulopathy

Collapsing glomerulopathy is  now recognized as a common, distinct pattern of proliferative parenchymal injury with poor response to empiric therapy. Histologically, it is characterized by partial or global sclerosis of the glomerular tuft along with hyperplasia and hypertrophy of the overlying visceral epithelial cells (podocytes), leading to the  formation of “ pseudocrescents”. VAryind degree of tubulointerstitial damge is associated with the glomerular changes.

The first description of the disease appeared in 1978 and was named “malignant focal segmental glomerulosclerosis” (FSGS) because of rapidly progressive nephrotic syndrome.Initially it was thought to be a specific pattern of glomerular injury associated with HIV infection (HIV associated nephropathy). In 1986, Weiss et al. described a similar renal lesion in HIV-negative patients with severe proteinuria and rapid progression to renal failure, and the term “collapsing glomerulopathy” was used for the first time to indicate this“new clinical-pathologic entity.”
The recently prosposed “Colombia” classification of focal and segmental glomerular sclerosis identifies collapsing glomerulopathy as a variant of FSGS.
Currently an increasing number of clinical associations are being recognized with this pattern of injury.
 The reader is referred to two excellent and comprehensive reviews which address the basic pathophysiology, as well as recent understanding of this disease.

http://jasn.asnjournals.org/cgi/reprint/17/10/2854

http://jasn.asnjournals.org/cgi/rapidpdf/ASN.2007080926v1.pdf