Collapsing FSGS: a clinicopathologic study of 10 cases from Pakistan

Abstract

Idiopathic collapsing FSGS is increasingly being reported in patients who have no HIV infection or history of IV drug abuse. There is a need for increased awareness among pathologists all over the world to diagnose this condition to guide nephrologists and patients regarding the poor prognosis of this form of FSGS.

Collapsing Glomerulopathy: The Expanding Etiologic Spectrum of a Shrinking Glomerular Lesion

“…Although the first report of CG included only six patients, this was soon followed by a series of fairly large studies from different centers mostly from United States, and subsequently from Europe and more recently from the developing countries (Agarwal et al, 2008;Bariety et al, 1998;Deegans et al, 2008;Detwiler et al, 1994;Grcevska & Polenakovik, 1999;Kazi & Mubarak, 2007;Laurinavicious et al, 1999;Meyrier, 1999;Mubarak & Kazi, 2010;Nada et al, 2009;Valeri et al, 1996;Weis et al, 1986). However, a wide variation is noted in the reported frequency of the lesion among all cases of FSGS.…”

“…The exact cause for the increase in the incidence of CG is not known, but may be due to a possible change in the exposure to certain infective organisms, chemical agents, or other environmental factors. Similarly, high rates of detection have been reported both in native and transplanted kidneys from developing countries, but no formal upward trend analysis of CG has been reported from developing countries till date (Gupta et al, 2011;Kazi & Mubarak, 2007;Mubarak & Kazi, 2010;Nada et al, 2009). …”

“…On presentation, a majority (>80%) of patients with CG have nephrotic range proteinuria, and studies have documented a significantly greater frequency of nephrotic syndrome (NS) and higher levels of proteinuria in patients with CG compared with patients with non-collapsing FSGS. There is also significantly greater renal functional impairment in patients with CG at presentation than patients with non-collapsing FSGS (Schwimmer et al, 2003 Once a diagnosis of CG is rendered, the possibility of HIVAN must be ruled out, usually by negative HIV serological tests, corroborated by the absence of endothelial tubuloreticular inclusions on ultrastructural examination (Mubarak & Kazi, 2010).…”

“…Patients with CG are at high risk of progressing to ESRD. Even with the currently available treatment, the incidence of ESRD is 50% to 100% in most series (Agarwal et al, 2008;Deegans et al, 2008;Detwiler et al, 1994;Grcevska & Polenakovik, 1999;Kazi & Mubarak, 2007;Laurinavicious et al, 1999;Meyrier, 1999;Mubarak & Kazi, 2010;Nada et al, 2009;Valeri et al, 1996;Weis et al, 1986). In all comparative studies, the renal survival of patients with CG was significantly worse than patients with non-collapsing FSGS.…”

“…Although most of the early studies were reported in the native kidneys, the disease has also been found more recently to afflict the transplanted kidneys, either as a recurrent or de novo disease, frequently leading to loss of the allograft (Schwimmer et al, 2003;Canaud et al, 2010;Gupta et al, 2011;Nadasday et al, 2002;Stokes et al, 1996;Swaminathan et al, 2006). Most of the cases have been reported from the western countries, but the lesion is also being increasingly recognized in the tropical regions www.intechopen.com Topics in Renal Biopsy and Pathology 140 (Kazi & Mubarak, 2007;Mubarak & Kazi, 2010;Nada et al, 2009). The recent increase in the reporting of CG partly reflects a genuine increase in the incidence of the disease and partly a detection bias resulting from heightened recognition by the pathologists .…”