Role of vWF/ADAMTS13 ratio and vWF function in progression of immunoglobulin A nephropathy

doi:10.3969/j.issn.1673-8640.2026.01.001

Abstract

Abstract:

Objective To investigate the role of the ratio of von Willebrand factor（vWF）to a disintegrin and metalloproteinase with a thrombospondin type 1 motif 13（ADAMTS13）and vWF function in evaluating the disease progression of immunoglobulin A nephropathy（IgAN）patients. Methods A total of 84 IgAN patients and 79 healthy subjects（healthy control group）from Ruijin Hospital of Shanghai Jiao Tong University School of Medicine were enrolled from December 2023 to February 2024. According to the Kidney Disease：Improving Global Outcomes（KDIGO）guidelines，the IgAN patients were classified into low-risk group（32 cases），intermediate-risk group（32 cases）and high-risk group（20 cases）. The general information and laboratory routine determination results were collected，and plasma vWF antigen（vWF：Ag），vWF collagen binding（vWF：CB），ADAMTS13 activity and vWF multimers were determined. The efficacy of each indicator in determining the high-risk status of IgAN patients was evaluated by receiver operating characteristic（ROC） curve. Results Compared with healthy control group，the levels of vWF：Ag，vWF：CB，vWF：Ag/ADAMTS13 ratio and vWF：CB/ADAMTS13 ratio in low-risk group，intermediate-risk group and high-risk group were increased（P<0.001）. Compared with healthy control group，the percentages of low-molecular-weight von Willebrand factor multimers（LMWM-vWF）and intermediate-molecular-weight von Willebrand factor multimers（IMWM-vWF）in high-risk group were increased（P<0.01），and the percentage of high-molecular-weight von Willebrand factor multimers（HMWM-vWF）and ADAMTS13 activity were decreased（P<0.05）. The levels of vWF：Ag and vWF：CB in high-risk group were higher than those in low-risk group（P<0.05），and the vWF：Ag/ADAMTS13 ratio and vWF：CB/ADAMTS13 ratio were higher than those in low-risk group（P<0.05）. The areas under curves（AUC）of vWF：Ag/ADAMTS13 ratio，vWF：CB/ADAMTS13 ratio，serum creatinine（SCr）and vWF：Ag in predicting the high-risk status of IgAN patients were 0.731，0.752，0.952 and 0.716，respectively. Conclusions The levels of vWF and ADAMTS13 in IgAN patients are abnormal，and the increase in vWF/ADAMTS13 ratio is related to the progression of kidney disease.

Key words: Von Willebrand factor, A disintegrin and metalloproteinase with a thrombospondin type 1 motif 13, Immunoglobulin A nephropathy

CLC Number:

R446.11

WU Zhuolin, YUAN Junwei, ZHANG Ziqi, LIU Yu, XU Guanqun, WANG Xuefeng, LU Yeling. Role of vWF/ADAMTS13 ratio and vWF function in progression of immunoglobulin A nephropathy[J]. Laboratory Medicine, 2026, 41(1): 1-7.

Figures/Tables 7

References 28

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组别	例数	性别		年龄/岁	免疫抑制治疗/例	血栓事件/例	APTT/s	PT/s	WBC计数/ （×109L^-1）	RBC计数/ （×109L^-1）	Hb/ （g·L^-1）
组别	例数	男/例	女/例	年龄/岁	免疫抑制治疗/例	血栓事件/例	APTT/s	PT/s	WBC计数/ （×109L^-1）	RBC计数/ （×109L^-1）	Hb/ （g·L^-1）
正常对照组	79	38	41	44.80±15.87	0	0	28.92±2.09	11.55±0.64	5.98±1.31	4.63 ±0.46	140.68±13.78
低危组	32	16	16	37.69±12.07	7	0	28.59±1.72	11.41±0.58	7.12±2.35*∆	4.62 ±0.68	138.19±19.79
中危组	32	20	12	47.03±15.07	9	0	28.73±2.11	11.45±0.66	6.49±1.58	4.17 ±0.57*#	128.25±18.72*#
高危组	20	8	12	45.10±13.19	6	2	27.69±1.73	11.16±0.80	8.07±2.40*∆	3.39±1.05*#∆	104.80±22.36*#∆
统计值		2.908		2.520	0.520	5.899	2.088	1.998	8.894	23.969	24.798
P值		0.406		0.060	0.771	0.052	0.104	0.116	<0.001	<0.001	<0.001
组别	PLT计数/ （×109L^-1）			ALT/ （IU·L^-1）	AST/ （IU·L^-1）		SCr/ （μmol·L^-1）	UA/ （μmol·L^-1）	DD/ （mg·L^-1）		eGFR/ [mL·（min·1.73 m2）-1]
正常对照组	239.15±53.93			21.24±12.73	21.49±5.11		72.0（59.0~84.0）	319.25±76.55
低危组	233.50±48.59			19.94±14.03	21.19±8.69		81.5（70.3~98.5）	337.69±87.92	0.53±0.61		90.21±17.44
中危组	200.49±69.94*			14.53±5.02	19.97±4.48		150.5（117.3~178.5）*#	365.84±95.50*	0.46±0.55		45.72±10.52#
高危组	230.50±96.58			20.50±15.93	19.45±6.00		303.0（193.5~640.0）*#∆	376.90±41.66*	1.19±1.61#∆		17.86±9.75#∆
统计值	2.934			2.296	0.936		107.910	4.327	4.248		193.052
P值	0.035			0.080	0.425		<0.001	0.006	0.018		<0.001

组别	例数	性别		年龄/岁	免疫抑制治疗/例	血栓事件/例	APTT/s	PT/s	WBC计数/ （×109L^-1）	RBC计数/ （×109L^-1）	Hb/ （g·L^-1）
组别	例数	男/例	女/例	年龄/岁	免疫抑制治疗/例	血栓事件/例	APTT/s	PT/s	WBC计数/ （×109L^-1）	RBC计数/ （×109L^-1）	Hb/ （g·L^-1）
正常对照组	79	38	41	44.80±15.87	0	0	28.92±2.09	11.55±0.64	5.98±1.31	4.63 ±0.46	140.68±13.78
低危组	32	16	16	37.69±12.07	7	0	28.59±1.72	11.41±0.58	7.12±2.35*∆	4.62 ±0.68	138.19±19.79
中危组	32	20	12	47.03±15.07	9	0	28.73±2.11	11.45±0.66	6.49±1.58	4.17 ±0.57*#	128.25±18.72*#
高危组	20	8	12	45.10±13.19	6	2	27.69±1.73	11.16±0.80	8.07±2.40*∆	3.39±1.05*#∆	104.80±22.36*#∆
统计值		2.908		2.520	0.520	5.899	2.088	1.998	8.894	23.969	24.798
P值		0.406		0.060	0.771	0.052	0.104	0.116	<0.001	<0.001	<0.001
组别	PLT计数/ （×109L^-1）			ALT/ （IU·L^-1）	AST/ （IU·L^-1）		SCr/ （μmol·L^-1）	UA/ （μmol·L^-1）	DD/ （mg·L^-1）		eGFR/ [mL·（min·1.73 m2）-1]
正常对照组	239.15±53.93			21.24±12.73	21.49±5.11		72.0（59.0~84.0）	319.25±76.55
低危组	233.50±48.59			19.94±14.03	21.19±8.69		81.5（70.3~98.5）	337.69±87.92	0.53±0.61		90.21±17.44
中危组	200.49±69.94*			14.53±5.02	19.97±4.48		150.5（117.3~178.5）*#	365.84±95.50*	0.46±0.55		45.72±10.52#
高危组	230.50±96.58			20.50±15.93	19.45±6.00		303.0（193.5~640.0）*#∆	376.90±41.66*	1.19±1.61#∆		17.86±9.75#∆
统计值	2.934			2.296	0.936		107.910	4.327	4.248		193.052
P值	0.035			0.080	0.425		<0.001	0.006	0.018		<0.001

组别	例数	LMWM-vWF/%	IMWM-vWF/%	HMWM-vWF/%
正常对照组	20	14.7（13.3~17.6）	30.8（29.0~31.8）	53.7（51.0~56.4）
低危组	4	17.8（17.3~20.3）	32.4（30.0~33.9）	50.3（46.1~52.3）
中危组	4	19.4（17.6~23.1）	33.5（30.7~34.2)	48.2（44.6~48.8）
高危组	4	25.9（23.1~28.5）*	36.4（33.7~37.4）*	37.8（36.6~40.7）*
统计值		12.75	9.50	16.07
P值		0.005	0.023	0.001

组别	例数	LMWM-vWF/%	IMWM-vWF/%	HMWM-vWF/%
正常对照组	20	14.7（13.3~17.6）	30.8（29.0~31.8）	53.7（51.0~56.4）
低危组	4	17.8（17.3~20.3）	32.4（30.0~33.9）	50.3（46.1~52.3）
中危组	4	19.4（17.6~23.1）	33.5（30.7~34.2)	48.2（44.6~48.8）
高危组	4	25.9（23.1~28.5）*	36.4（33.7~37.4）*	37.8（36.6~40.7）*
统计值		12.75	9.50	16.07
P值		0.005	0.023	0.001

项目	AUC（95%CI①）	最佳临界值	敏感性/%	特异性/%	Youden指数
vWF：Ag/ADAMTS13比值	0.731（0.618~0.843）	0.291	80.0	57.6	0.424
vWF：CB/ADAMTS13比值	0.752（0.645~0.860）	0.399	80.0	68.3	0.482
SCr	0.952（0.901~0.977）	155.00 μmol·L^-1	95.0	83.5	0.835
vWF：Ag	0.716（0.596~0.836）	161.12%	90.0	47.1	0.371