New antigens involved in membranous nephropathy beyond phospholipase A2 receptor

Table 1 Clinical findings in Semaphorin 3B-associated membranous nephropathy[8,28,29]

Case	Age (yr)/sex	UP (g/24)	Serum CR (mg/dL)	Remission	Serum CR/UP/24 h
1	41/F	7.9	0.74	Spontaneous	0.6/no proteinuria (16 mo)
2	26/F	6.2	0.4	Spontaneous	0.43/400 mg (18 mo)
3	2/M	5	0.5	Immunosuppressive	0.35/150 mg (24 mo)
4	40/F	17.3	0.9	Immunosuppressive	0.6/no proteinuria (10 yr)
5	19 mo/M	0.4	0.7	Immunosuppressive	0.9/400 mg (18 mo)
6	2/F	UP/CR ratio 6.81	0.21	Immunosuppressive	UP/CR ratio 0.23 (13 mo)
7	17/M	UP/CR ratio 0.78	0.6	Immunosuppressive	UP/CR ratio 0.1 (19 mo)
8	9 mo/M	UP/CR ratio 0.94	0.45	Immunosuppressive	UP/CR ratio 0.09 /14 yr)
9	2/M	UP/CR ratio 1.95	0.13	Immunosuppressive	UP/CR ratio 0.12 (5 yr)
10	14/M	3	0.64	Lost to follow up	n/a
11	16/M	12	0.83	Immunosuppressive	Dialysis

F: Female; M: Male; UP: Urinary protein; CR: Creatinine.

Table 2 Clinical and immunomicroscopy findings in protocadherin patients[35,36]

Patient	Age (yr)	Urinary protein (g/24h)	Serum creatinine (mg/dL)	Immunofluorescence	IgG
1	73	3.2	1.1	IgG 3+; C1q 1+	IgG1 2+; IgG3 1+; IgG4 1+
2	66	9.6	1.3	IgG 2+	IgG1 1+; IgG4 2+
3	68	NA	1.1	IgG 2+	IgG4 2+
4	59	3	1.1	IgG 2+; C3 1+	1gG 1+; IgG2 2+; IgG3 2+; IgG4 3+
5	61	7	1.9	IgG 3+; C3 1+	IgG1 2+; IgG3 2+
6	38	3	1	IgG 1+	NA
7	37	1.4	1.76	IgG3 +	IgG4 1+
8	67	4.3	1.2	IgG 3+	NA
9	75	7	1	IgG 3+; C3 1+	NA
10	70	1	1.6	IgG 3+; C3 1+	NA
11	64	8.4	1.2	IgG 3+; C3 3+	IgG3 2+; IgG2 2+
12	61	3.9	1	IgG 3+	IgG4 2+; IgG2 1+
13	66	23.3	3.8	IgG 3+; IgA 2+	IgG4 2+; IgG2 2+
14	72	21	1.3	IgG 1+	NA

NA: Not applicable. IgG: Immunoglobulin G; IgA: Immunoglobulin A.

Table 3 Demographic and clinical characteristics according to antigen

Total 270	PLA2R 220	EXT1/EXT2 11	NELL 1 6	PCDH7 3	THSD7A 1	NCAM-1 1	Septule negative 28
Age	54.0 (43.2-61.0)	40.0 (25.0-48.0)	57.0 (36.7-66.5)	73 (69.0-74.0)	67.0	46	52 (44.5-66.5)
Male sex % (n)	75.0 (165/220)	27.2 (3/11)	66.6 (4/6)	33.3 (1/3)	100 (1/1)	0 (0/1)	60.7 (17/28)
Serum creatinine (mg/dL)	1.1 (0.9-1.4)	1.0 (0.7-1.1)	1.9 (1.0-4.9)	1.1 (1.03-1.48)	0.9	0.6	1.2 (0.9-1.7)
eGFR (mL/min/1.73 m2)	68 (49.9-91.0)	85.0 (65.2- 113.4)	38.3 (14.5-75.7)	57 (39.8-66.6)	89.5	114.0	67 (40.0-95.0)
Proteinuria (g/24 h)	8.0 (5.2-12.0)	5.6 2.6-9.3)	11.0 (6.8-16.1)	3.2 ( 1.55-6.05)	14.4	5.7	4.5 (3.2-9.9)
Malignancy %	5.0 (11/220)	9.0 (1/11)	33.3 (2/6)	-	100 (1/1)	-	25.0 (7/28)
Autoimmunity %	5.4 (12/220)	81.8 (9/11)	33.3 (2/6)	-	-	-	46.4 (13/28)
Paraproteinmemia %	4.0 (9/220)	-	-	-	100 (1/1)	-	35 (1/28)
Infection %	0.4 (1/220)	-	-	-	-	-	-
NSAID %	1.8 (4/220)	-	16.6 (1/6)	-	-	-	14.2 (6/28)
No associated disease %	84.0 (185/220)	18.1 (2/11)	16.6 (1/6)	100 (3/3)	-	100 (1/1)	21.4 (6/28)

eGFR: Estimated glomerular filtration rate; EXT: Exostosin; NCAM-1: Neural cell adhesion molecule 1; NELL-1: Neural epidermal growth factor-like 1 protein; NSAID: Nonsteroidal anti-inflammatory drug; PCDH7: Protocadherin; PLA2R: Phospholipase A2 receptor; THSD7A: Thrombospondin type 1 domain containing 7A.