Collapsing FSGS: a clinicopathologic study of 10 cases from Pakistan.
BACKGROUND
Idiopathic collapsing focal segmental glomerulosclerosis (FSGS), a rare variant of FSGS, is of interest because of its increasing incidence, frequent association with black race, HIV-1 infection, and intravenous (IV) drug abuse. This lesion has not been reported from Pakistan until now.


METHODS
We reviewed our 14-year native renal biopsies record and identified 10 cases of this entity (July 1995-July 2009). Patients' demographic and clinicopathologic data were collected from case files. Renal biopsies were studied by light microscopy, immunofluorescence microscopy, and electron microscopy. A control group of 124 patients with noncollapsing FSGS was selected for comparison of clinical, laboratory, and outcome parameters.


RESULTS
All the patients were young adults (mean age: 22.4 +/- 4.6 years). The majority were males (9 vs. 1 female). All presented with nephrotic syndrome (24-h urinary protein: 6.7 +/- 9.22 g). In addition, 8 had mild to moderate renal failure (serum creatinine: 4.12 +/- 4.6 mg/dl). No history of heroin or IV drug abuse was elicited and all tested negative for HIV. Only two patients (20%) responded to steroid treatment, while eight (80%) did not. Five of the patients (50%) developed endstage renal disease after a mean interval of 18 months.


CONCLUSIONS
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.
Histopathology Department, Sindh Institute of Urology and Transplantation, Karachi, 74200, Pakistan. drmubaraksiut@yahoo.com
Muhammed
M
Mubarak
Javed I
JI
Kazi
D000293:Adolescent; D000328:Adult; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D009404:Nephrotic Syndrome / Q000209:etiology; D010154:Pakistan / Q000453:epidemiology; D012189:Retrospective Studies