Efficacy of extracorporeal plasma therapy for adult native kidney patients with Primary FSGS: a Systematic review

Figures & data

Figure 1. PRISMA flow diagram for study selection.

Table 1. Modality, session and duration of EPT and renal outcomes in primary FSGS.

Modality of EPT	Study, first author, year (reference)	EPT total sessions	EPT duration	Follow up duration	Responders, n/total (%)	CR, n/total (%)	PR, n/total (%)
PE (n = 31 patients)
5 case reports	Ginsburg, 1997 [28]	35	2 years	12 months	1		1
	Ishii, 2002 [23]	4	2 weeks	6 months	1		1
	Oliverira, 2007 [25]	7	3 weeks	24 months	1	NA	NA
	Cader, 2017 [29]	88	1.5 years	18 months	1	1
	Schenk, 2017 [22]	NA	NA	12 months	1		1
3 case series	Feld, 1998 [18]	6	2 weeks	29 ± 4 months	2/8 (25) Long term 1/8 (13)		2/8 (25) Long term 1/8 (13)
	Mitwalli, 1998 [17]	17	6 months	27.5 ± 6.3 months	8/11 (73) Long term 6/11 (55)	6/11 (55) (Long term)	2/11 (18)
	Dirim, 2022 [31]	14 (IQR 10, 23)	>6 weeks	17 months (IQR 15, 20)	5/7 (72) Long term 3/7 (43)	1/7 (14) (Long term)	4/7 (58) Long term 2/7 (29)
LDL-A (n = 61 patients)
3 case reports	Yorioka, 1997 [27]	8	4 weeks	3 months	1		1
	Araki, 2015 [26]	9	4.5 weeks	∼4 months	1	NA	NA
	Yamazaki, 2016 [21]	11	5.5 weeks	3 weeks	1	NA	NA
3 case series	Muso, 2015 [30]	average 9.6	NA	4 weeks	14/26 (54) ^a	NA	NA
	Muso, 2015 [34]	9.6 ± 2.7	NA	2 years	12/28 (43) (Long term)	12/28 (43) (Long term)
	Muso, 1999 [35]	NA	NA	2 weeks	4/7 (57)	NA	NA
IA (n = 10 patients)
1 case report	Kuhn, 2006 [24]	10	4 weeks	NA	1		1
2 case series	Haas, 1998 [33]	5	10 days	NA	2/5 (40)		2/5 (40)
	Moriconi, 2001 [32]	10	4 weeks	6 months	1/4 (25)		1/4 (25)
LCAP (n = 2 patients)
1 case series	Yokoyam, 2002 [36]	4	2 weeks	47 and 40 months	1/2 (50)	1/2 (50)

EPT: extracorporeal plasma therapy; FSGS: focal segmental glomerulosclerosis; CR: complete remission; PR: partial remission; PE: plasmapheresis or plasma exchange using albumin; NA: not available; IQR: interquartile (25% and 75% percentile); LDL-A: LDL apheresis using dextran sulfate cellulose column; IA: immunoadsorption using protein A or IgG; LCAP: lymphocytapheresis using Cellsorba, a leukapheresis filter. ^a 26 episodes of LDL-A in 23 patients.

Table 2. General characteristics of patients with primary FSGS.

Patients (n)	Male, n/total (%)	Age (y)
Non-hemodialysis (101)	18 M; 24 F
Hemodialysis (3)	1 M; 2 F	74; 43 and 81
Total (104)	19/45 (42) NA in 3 studies (n = 8, 23 and 28)	9 case report studies and a case series study (n = 11): 39.7 ± 20.3; 34 (IQR 24, 45) ^a 8 case series studies Haas [33]: n = 5; 28.4 ± 12.4; 25 (IQR 19.5, 39) Mitwalli [17]: n = 11; 32 ± 6.6 Dirim [31]: n = 7; 26.4 ± 9.8; 23 (IQR 19, 32) Muso [35]: n = 7; 34 ± 13.8; 26 (IQR 22, 46) Moriconi [32]: n = 4: range 18–60 NA in 3 case series (n = 8, 23 and 28) [18,30,34]

FSGS: focal segmental glomerulosclerosis; M: male; F: female; NA: not available; IQR: interquartile (25% and 75% percentile). ^a9 case report studies [21–29]. The case series study only had two cases with FSGS [36].

Table 3. Overall renal outcomes following EPT therapy in primary FSGS.

Patients (n)	Responders, n/total (%)	CR, n/total (%)	PR, n/total (%)
Non-hemodialysis (101)	55/101 (54)	21/71 (30)^a	16/71 (23)^a
Hemodialysis (3)	3/3 (100)
Total (104)	58/104 (56) Long-term 30/65 (46)^b

EPT: extracorporeal plasma therapy; FSGS: focal segmental glomerulosclerosis; CR: complete remission; PR: partial remission. ^aNot available in 2 case series studies (n = 7 and 23, respectively). Remission and effectiveness were reported in these 2 studies. ^bLong-term indicates follow-up duration is ≥6 months.

Table 4. Summary of renal outcomes following different EPT in primary FSGS.

Patients (n)	Responders, n/total (%)	CR, n/total (%)	PR, n/total (%)	Follow up duration
PE (31)	20/31 (65) Long-term 15/31 (48)^a			14.4 ± 6.8 months in 5 case report studies [22,23,25,28,29] 29 ± 4 months in a case series study [18] 27.5 ± 6.3 months in a case series study [17] 17 months (IQR 15, 20) in a case series study [31]
Non-HD (30)	19/30 (63)	8/30 (27)	11/30 (37)
HD (1)	1/1 (100)
LDL-A (61)	33/61 (54) Long-term 12/28 (43)			3 weeks, 3 months and 4 months in 3 case report studies, respectively [21,26,27] 2 week, 4 weeks and 2 years in 3 case series studies, respectively [30,34,35]
Non-HD (59)	31/59 (53)	12/29 (41)^b	1/29 (3)^b
HD (2)	2/2 (100)
IA (10)^c	4/10 (40) Long-term ¼ (25)	0/10 (0)	4/10 (40)	25 weeks and 6 months in 2 patients, respectively [32] NA in 7 patients
LCAP (2)^c	1/2 (50) Long-term 1/2 (50)	1/2 (50)		47 and 40 months, respectively [36]

EPT: extracorporeal plasma therapy; FSGS: focal segmental glomerulosclerosis; CR: complete remission; PR: partial remission; IQR: interquartile (25% and 75% percentile); PE: plasmapheresis or plasma exchange using albumin; HD: hemodialysis; LDL-A: LDL apheresis using dextran sulfate cellulose column; IA: immunoadsorption using protein A or IgG; NA: not available; LCAP: lymphocytapheresis using Cellsorba, a leukapheresis filter.

^aLong-term indicates follow-up duration is ≥6 months. ^bNA in 2 case series studies (n = 7 and 23, respectively). Remission and effectiveness were reported in the 2 studies [30,35]. ^cThe two patients are non-hemodialysis.

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