Cellular focal segmental glomerulosclerosis: Clinical and pathologic features.
Five pathologic variants of idiopathic focal segmental glomerulosclerosis (FSGS) are recognized: collapsing (COLL), cellular (CELL), glomerular tip lesion (GTL), perihilar, and not otherwise specified (NOS). The prognostic significance of CELL FSGS has not been determined. We compared the presenting clinical and pathologic characteristics in 225 patients with CELL (N=22), COLL (N=56), GTL (N=60), and NOS (N=87) variants of idiopathic FSGS. CELL, COLL, and tip lesion all showed greater frequency and severity of nephrotic syndrome, and shorter time to biopsy compared to NOS. Predictors of end-stage renal disease (ESRD) for all FSGS patients included initial serum creatinine, % global sclerosis, % COLL lesions, chronic tubulo-interstitial injury score, and lack of remission response. COLL FSGS had the highest rate of renal insufficiency at presentation, most extensive glomerular involvement and chronic tubulo-interstitial disease, fewest remissions (13.2%), and highest rate of ESRD (65.3%). GTL patients were older and showed the highest remission rate (75.8%) and lowest rate of ESRD (5.7%). CELL variant showed intermediate rates of remission (44.5%) and ESRD (27.8%) compared to COLL and tip lesion. CELL variant may include cases of unsampled tip or COLL lesion, underscoring the importance of adequate sampling. Our data support the view that CELL and COLL FSGS are not equivalent and validates an approach to pathologic classification that distinguishes between COLL, CELL, and tip lesion variants of FSGS.
Stokes
M B
MB
Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York, USA. mbs2101@columbia.edu
Valeri
A M
AM
Markowitz
G S
GS
D'Agati
V D
VD
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D015331:Cohort Studies; D003404:Creatinine / Q000097:blood; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000145:classification* / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007676:Kidney Failure, Chronic / Q000097:blood / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D007678:Kidney Glomerulus / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D050199:Podocytes / Q000473:pathology*; D011237:Predictive Value of Tests; D051437:Renal Insufficiency / Q000473:pathology / Q000503:physiopathology; D012189:Retrospective Studies; D012598:Sclerosis; D012720:Severity of Illness Index; D016896:Treatment Outcome
D003404:Creatinine