Table 1:

Clinical and immunological features of 165 patients with C3 Glomerulopathy including 64 children and 101 adults

	Nb	All patients 165	Children 64	Adults 101
Clinical data at diagnosis
Sex (% of male)	165	82 (50%)	25 (39%)	57 (56%)	0.01
Age (years)	165	20 [10–39]	8[6–11]	33[21–54]	<0.0001
Proteinuira g/day	96	3,1 [1.9–6.4]	3[1.6–5.4]	3.5[2.3–6.6]	0.72
Nephrotic range proteinuria	143	67 (47%)	23/56 (41%)	34/85 (40%)	0.13
Estimated GFR (ml/min/1.73 m2)	151	65 [28–100]	80 [40–100]	56 [28–100]	0.11
Stage 3–5 CKD n (%)	151	71 (47%)	25/61 (41%)	46/90 (51%)	0.22
Immunological data at diagnosis
C3 (660 to 1250 mg/L)	165	557 [257–879]	443[192–847]	578[311–1680]	0.08
Low C3 (<660 mg/L) (%)	165	97 (59%)	40/64 (63%)	57/101 (56%)	0.51
Soluble C5b-9 (ng/mL)	165	482 [310–849]	488[323–868]	482[289–847]	0.72
High sC5b-9 (>460ng/mL) (%)	165	84 (51%)	33 (52%)	51 (50%)	0.99
C4 (93 to 380 mg/L)	154	226[169–286]	222[151–274]	234[179–296]	0.42
Factor H level (% of normal)	144	105 [82–124]	104[82–126}	105[83–123]	0.85
Factor H (<65% of the normal) (%)	142	6 (6%)	1/50 (2%)	5/92 (5%)	0.33
Factor I level (% of the normal)	142	109[95–124]	114[99–130]	105[92–120]	0.12
Low Factor I (<) (%)	142	4 (3%)	2/50 (4%)	2/92 (2%)	0.52
C3NeF n (%)	161	90 (56%)	50/64 (78%)	40/99 (40%)	<0.0001
C5NeF n (%)	92	35 (38%)	20/43 (47%)	15/51 (29%)	0.13
Anti-FH auto-antibodies n (%)	154	22 (14%)	11/56 (19%)	11/98 (11%)	0.15
rare/pathogenic variant	140	26 (19%)	9/53 (17%)	17/87 (20%)	0.82
Follow-up
Median Follow-up (months)	162	47 [19–82]	60 [20–99]	36[16–75]	0.02
ESKD n (%)	162	46 (28%)	11/62 (18%)	35/100 (35%)	0.02
Duration of evolution until ESKD (months)		22 [9–77]	30[18–88]	21[6–66]	0.33

Abbreviations:, CKD, chronic kidney disease, ESKD, end stage kidney disease; eGFR, Estimated Glomerular Filtration Rate, FH, factor H, sC5b9: soluble C5b9

Results are expressed as median plus interquartile (IQR).