A prospective study of collapsing focal segmental glomerulosclerosis

Figures & data

Figure 1. Photomicrographs of cFSGS show (a) global collapse of glomerular tuft wish hyperplasic and hypertrophied podocytes with overlying hypertrophied and hyperplastic podocytes (b) Hyperplastic podocytes (Ki 67 positive inset) over segmental collapsed tuft show mitosis, moderate cytoplasm with protein absorption droplets and prominent nucleoli (c) Segmental collapse with collagenized glomerular tuft having a crown of quiescent podocytes (d) Global collapse lined by row of cuboidal podocytes without mitosis and protein absorption droplets (PAS- Silver Methenamine stain a, c–d, Masson’s Trichrome-b, Immunohistochemistry Ki 67-insetd, a–c, X 40, d-X 20 original magnification).

Table 1. Comparison of clinical and pathological parameters of cFSGS and steroid and tacrolimus resistant FSGS.

	cFSGS (n–22)	Resistant FSGS (n–19)	p values
Age (years)	26.9 ± 9.4 (14–55)	28.1 ± 8.6 (18–43)	0.67
Sex (Male/Female)	15/07	15/04	0.49
Proteinuria (gm/day)	4.6 ± 3.0 (1.2–13)	4.5 ± 3.8 (1.2 ± 15.2)	0.93
Serum creatinine (mg/dl)	4.1 ± 3.7 (0.7–12.2)	1.4 ± 0.7 (0.7–1.1)	0.003
Serum albumin (gm/dl)	2.1 ± 0.6 (1.2–3.6)	2.0 ± 0.5 (0.64–4.5)	0.63
Hypertension	17(77.2%)	06(31.5%)	0.004
ESRD/Death	12 (54.5%)	05 (15.7%)	0.11
Progressive CKD	04 (18.1%)	05 (15.7%)	0.70
Percentage collapse (%)	48.0 ± 33.7 (4.5–100)	None	<0.0001
IFTA (≥2+)	8 (36.3%)	None	0.004
Tubulocystic inclusion	09 (40.9%)	None	0.001
TMA	08 (36.3%)	None	0.004
Arteriosclerosis	04 (18.2%)	None	0.11
Ki67	15 (68.1%)	None	<0.0001
WT1	22 (100%)	None	<0.0001
PAX2	13 (59%)	None	<0.0001
Parvo B19	08 (36.3%)	03 (15.7%)	0.17

Notes: cFSGS—collapsing focal segmental glomerulosclerosis, FSGS—focal segmental glomerulosclerosis, Resistant—no remission with 16 weeks of oral prednisolone followed by 24 weeks of tacrolimus with steroids, ESRD—end-stage renal disease, CKD—chronic kidney disease, IFTA—interstitial fibrosis and tubular atrophy, TMA—thrombotic microangiopathy.

Table 2. Brief summary of all the patients with cFSGS.

S No	Age/Sex	Syndrome	Microscopic Hematuria	UP (gm/day)	S Creatinine (mg/dl)	Death	Treatment	Outcome (Duration)	Time to ESRD (Months)
1	27/M	NS	No	2.9	1.3	Yes	GC	Death (2mts)	NA
2	30/F	NS	Yes	1.86	1.78	Yes	GC	Death (2 mts)	NA
3	30/M	NS	No	3.6	0.7	No	GC, TAC	SDNS (28mts)	NA
4	26/M	NS	No	6.2	0.8	No	GC	IFR (60 mts)	NA
5	20/M	NS	No	6.3	0.8	No	GC, TAC, CTX, RTx	SRNS (60mts)	NA
6	30/F	NS	No	6.5	1.2	No	GC, TAC	SRNS (24 mts)	NA
7	19/M	NS	No	4.3	0.8	No	GC + TAC	CKD (60 mts)	NA
8	18/M	NS	No	2.0	0.8	No	ARB	PR (24 mts)	NA
9	55/F	NS	No	4.0	1.5	No	GC	CKD (24 mts)	NA
10	21/F	NS	No	7.8	1.1	No	GC	ESRD (36 mts)	30
11	18/F	NS	No	13	1.4	No	GC	CKD (24 mts)	NA
12	18/M	PRF	Yes	1.2	5.3	Yes	GC	Death (1mon)	NA
13	14/M	PRF	Yes	3.07	3.6	Yes	GC	ESRD-Death (04 mts)	NA
14	19/M	PRF	No	1.2	7.39	Yes	GC	Death (02 mts)	NA
15	18/F	PRF	Yes	6.8	8.5	No	GC	ESRD (36 mts)	03
16	22/M	PRF	Yes	7.6	11	No	GC	ESRD (03 mts)	03
17	18/F	PRF	Yes	8.4	7.8	Yes^a	GC, CTX	ESRD (09 mts)	03
18	40/M	PRF	No	1.4	10.2	No	GC	ESRD (09 mts)	03
19	14/M	PRF	Yes	3.07	3.6	Yes	GC	ESRD-Death (4 mts)	03
20	36/F	PRF	Yes	6.3	5.8	Yes	GC	ESRD-Death (12 mts)	03
21	30/M	PRF	Yes	4.2	4.8	Yes	GC	ESRD (05 mts)	05
22	18/M	PRF	No	3.0	2.73	No	GC	SRNS (24mts)	NA

Notes: cFSGS—collapsing focal segmental glomerulosclerosis, UP–urine protein,

^aDisseminated candida infection, ESRD—end-stage renal disease, GC—oral prednisolone (1 mg/kg/day), CTX—cyclophosphamide, TAC—tacrolimus, RTx—Rituximab, ARB—angiotensin receptor blocker, IFR—infrequent relapse, CKD—chronic kidney disease, SRNS—steroid-resistant nephrotic syndrome, partial remission, CR—complete remission, PRF—progressive renal failure.