Clinical analysis of pediatric lupus nephritis patients with positive serum antineutrophil cytoplasmic antibody

doi:10.3877/cma.j.issn.1673-5250.2026.03.004

Objective

To explore the clinical manifestations, renal histopathological features, and prognosis of pediatric lupus nephritis (LN) patients with positive serum antineutrophil cytoplasmic antibody (ANCA).

Methods

A total of 53 newly diagnosed LN children who were hospitalized in the Department of Nephrology, Capital Center for Children′s Health, Capital Medical University between January 2015 and February 2024 were selected. Based on the serum ANCA results, the children were divided into study group (n=9, with positive serum ANCA) and control group (n=44, with negative serum ANCA). A retrospective analysis was conducted to collect clinical data on the two groups, including clinical manifestations, laboratory test results, renal histopathological characteristics, and prognosis. Statistical comparisons were performed using independent samples t-test, Mann-Whitney U test, chi-square test, and Fisher′s exact test. This study was approved by the Ethics Committee of the Capital Center for Children′s Health, Capital Medical University (Approval No. SHERLL2021052).

Results

①Among 53 children with LN, 19 were male and 34 were female, with onset age of (11.2±2.8) years. There were no statistically significant differences in general clinical data such as gender ratio and age of onset between the study group and control group (P>0.05). ②Compared with the control group, the study group had lower levels of hemoglobin (Hb) [79.0 g/L (68.0, 88.5 g/L) vs 102.0 g/L (84.3, 113.8 g/L), Z=2.90, P=0.003], and higher systemic lupus erythematosus disease activity index 2000 (SLEDAI-2000) scores [23.0 points (18.0, 30.0 points) vs 18.0 points (16.0, 20.8 points), Z=-2.55, P=0.010], as well as higher erythrocyte sedimentation rate (ESR) [(86.1±43.5) mm/h vs (38.7±27.7) mm/h, t=-4.22, P<0.001]. There were no statistically significant differences in anti double stranded DNA (dsDNA) antibody titers, complement 3 and 4 levels, and incidence of extrarenal organ involvement between the two groups (P>0.05). The study group had higher levels of urine protein, and the difference was statistically significant (Z=-2.11, P=0.034). There were no statistically significant differences between the two groups in pyuria, gross hematuria, incidence of renal insufficiency, serum creatinine (SCr), blood urea nitrogen (BUN), estimated glomerular filtration rate (eGFR), and the distribution of renal pathological classification (P>0.05). Compared with the control group, the study group had a higher incidence of glomerular necrosis and a higher proportion of cells and cellular fibrous crescents (P=0.012; Z=-2.16, P=0.030). ③The median follow-up time for the study group and control group were 42.0 months (21.5, 63.5 months) and 16.5 months (6.0, 36.8 months), respectively. The complete remission (CR) time in the study group and control group were 2.5 months (2.0, 5.8 months) and 4.5 months (2.3, 6.8 months), respectively, and the difference was not statistically significant (Z=-1.37, P=0.185), and there were no statistically significant differences in the CR rate, the recurrence rate of LN, and the proportion of progress to end-stage renal disease (ESKD) (P>0.05). There were no statistically significant differences in the sustained CR and survival (non-progression to ESKD) curves between the two groups of children (P>0.05).

Conclusions

Compared with LN children with negative serum ANCA, LN children with positive serum ANCA have higher LN activity, more severe anemia and more serious renal damage, requiring timely diagnosis and treatment. However, the relationship between serum ANCA and the long-term prognosis of kidney disease in LN children remains unclear.

Key words: Antibodies, antineutrophil cytoplasmic, Lupus nephritis, Lupus erythematosus, systemic, Pathology, clinical, Child

表1 研究组和对照组LN患儿SLEDAI-2000评分、基线免疫生化指标与肾外器官受累情况比较

组别	例数	SLEDAI-2000评分[分，M(Q₁，Q₃)]	抗dsDNA抗体[IU/mL，M(Q₁，Q₃)]	补体3[g/L，M(Q₁，Q₃)]	补体4[g/L，M(Q₁，Q₃)]	WBC[×10¹²/L，M(Q₁，Q₃)]	Hb[g/L，M(Q₁，Q₃)]
研究组	9	23.0(18.0，30.0)	800.0(357.0，800.0)	0.29(0.15，0.40)	0.03(0.03，0.08)	3.4(3.3，4.2)	79.0(68.0，88.5)
对照组	44	18.0(16.0，20.8)	570.5(325.3，800.0)	0.27(0.21，0.39)	0.05(0.03，0.08)	3.9(2.9，5.2)	102.0(84.3，113.8)
统计量		Z＝－2.55	Z＝－0.79	Z＝－0.05	Z＝－0.50	Z＝－0.92	Z＝2.90
P值		0.010	0.465	0.972	0.633	0.368	0.003
组别	例数	ESR(mm/h，±s)	PLT(×10⁹/L，±s)	皮肤黏膜受累[例数(%)]	呼吸系统受累[例数(%)]	消化系统受累[例数(%)]	心脏受累[例数(%)]	神经系统受累[例数(%)]	血液系统受累[例数(%)]
研究组	9	86.1±43.5	187.3±63.6	3(33.3)	4(44.4)	4(44.4)	1(11.1)	3(33.3)	9(100.0)
对照组	44	38.7±27.7	167.9±90.4	22(50.0)	5(11.4)	6(13.6)	5(11.4)	7(15.9)	40(90.9)
统计量		t＝－4.22	t＝－0.61	χ²＝0.30	χ²＝3.69^a	χ²＝2.84^a	χ²<0.01^a	χ²＝0.56^a	—^b
P值		<0.001	0.544	0.585	0.055	0.092	>0.999	0.453	1.000

表1 研究组和对照组LN患儿SLEDAI-2000评分、基线免疫生化指标与肾外器官受累情况比较

组别	例数	SLEDAI-2000评分[分，M(Q₁，Q₃)]	抗dsDNA抗体[IU/mL，M(Q₁，Q₃)]	补体3[g/L，M(Q₁，Q₃)]	补体4[g/L，M(Q₁，Q₃)]	WBC[×10¹²/L，M(Q₁，Q₃)]	Hb[g/L，M(Q₁，Q₃)]
研究组	9	23.0(18.0，30.0)	800.0(357.0，800.0)	0.29(0.15，0.40)	0.03(0.03，0.08)	3.4(3.3，4.2)	79.0(68.0，88.5)
对照组	44	18.0(16.0，20.8)	570.5(325.3，800.0)	0.27(0.21，0.39)	0.05(0.03，0.08)	3.9(2.9，5.2)	102.0(84.3，113.8)
统计量		Z＝－2.55	Z＝－0.79	Z＝－0.05	Z＝－0.50	Z＝－0.92	Z＝2.90
P值		0.010	0.465	0.972	0.633	0.368	0.003
组别	例数	ESR(mm/h，±s)	PLT(×10⁹/L，±s)	皮肤黏膜受累[例数(%)]	呼吸系统受累[例数(%)]	消化系统受累[例数(%)]	心脏受累[例数(%)]	神经系统受累[例数(%)]	血液系统受累[例数(%)]
研究组	9	86.1±43.5	187.3±63.6	3(33.3)	4(44.4)	4(44.4)	1(11.1)	3(33.3)	9(100.0)
对照组	44	38.7±27.7	167.9±90.4	22(50.0)	5(11.4)	6(13.6)	5(11.4)	7(15.9)	40(90.9)
统计量		t＝－4.22	t＝－0.61	χ²＝0.30	χ²＝3.69^a	χ²＝2.84^a	χ²<0.01^a	χ²＝0.56^a	—^b
P值		<0.001	0.544	0.585	0.055	0.092	>0.999	0.453	1.000

表2 研究组和对照组患儿LN相关临床表现比较

组别	例数	脓尿[例数(%)]	肉眼血尿[例数(%)]	肾功能不全[例数(%)]	尿蛋白水平[mg/(kg·d)，M(Q₁，Q₃)]	SCr[μmol/L，M(Q₁，Q₃)]	BUN[mmol/L，M(Q₁，Q₃)]	eGFR[mL/(min·1.73 m²), ±s]
研究组	9	8(88.9)	4(44.4)	6(66.7)	89.4(53.9，150.0)	66.2(59.0，79.4)	7.1(5.1，9.4)	83.0±24.4
对照组	44	31(70.5)	14(31.8)	26(59.1)	40.2(25.3，62.4)	62.4(46.0，87.1)	9.0(6.1，13.1)	88.5±36.4
统计量		χ²＝0.53	χ²＝0.12	χ²＝0.18	Z＝－2.11	Z＝－0.73	Z＝－1.08	t＝0.43
P值		0.467	0.732	0.672	0.034	0.476	0.293	0.670

表2 研究组和对照组患儿LN相关临床表现比较

组别	例数	脓尿[例数(%)]	肉眼血尿[例数(%)]	肾功能不全[例数(%)]	尿蛋白水平[mg/(kg·d)，M(Q₁，Q₃)]	SCr[μmol/L，M(Q₁，Q₃)]	BUN[mmol/L，M(Q₁，Q₃)]	eGFR[mL/(min·1.73 m²), ±s]
研究组	9	8(88.9)	4(44.4)	6(66.7)	89.4(53.9，150.0)	66.2(59.0，79.4)	7.1(5.1，9.4)	83.0±24.4
对照组	44	31(70.5)	14(31.8)	26(59.1)	40.2(25.3，62.4)	62.4(46.0，87.1)	9.0(6.1，13.1)	88.5±36.4
统计量		χ²＝0.53	χ²＝0.12	χ²＝0.18	Z＝－2.11	Z＝－0.73	Z＝－1.08	t＝0.43
P值		0.467	0.732	0.672	0.034	0.476	0.293	0.670

表3 研究组和对照组LN患儿肾组织病理学特征相关指标比较

组别	例数	LN起病至进行肾组织活检时间[d，M(Q₁，Q₃)]	LN病理学分型[例数(%)]			肾小球坏死[例数(%)]	间质纤维化[例数(%)]	肾小管萎缩[例数(%)]
组别	例数	LN起病至进行肾组织活检时间[d，M(Q₁，Q₃)]	Ⅱ型	Ⅲ型(Ⅲ＋Ⅴ型)	Ⅳ型(Ⅳ＋Ⅴ型)	肾小球坏死[例数(%)]	间质纤维化[例数(%)]	肾小管萎缩[例数(%)]
研究组	9	42.0(32.0，77.0)	0(0)	1(11.1)	8(88.9)	9(100.0)	7(77.8)	4(44.4)
对照组	44	28.0(21.0，65.7)	1(2.3)	4(9.1)	39(88.6)	25(56.8)	24(54.5)	10(22.7)
统计量		Z＝－1.54	—^a			—^b	χ²＝0.84^c	χ²＝0.87^c
P值		0.128	>0.999			0.012	0.359	0.352
组别	例数	细胞/纤维性新月体占比[%，M(Q₁，Q₃)]	细胞/纤维性新月体积分[分，M(Q₁，Q₃)]	肾小管萎缩＋间质纤维化积分[分，M(Q₁，Q₃)]	AI评分[分，M(Q₁，Q₃)]	CI评分[分，M(Q₁，Q₃)]	寡免疫复合物型LN[例数(%)]
研究组	9	26.7(13.0，35.0)	2(0，4)	0(0，0.5)	12.0(7.0，13.0)	0(0，1.5)	1(11.1)
对照组	44	8.0(0，21.5)	2(0，2)	0(0，0)	9.0(7.0，12.0)	0(0，1.0)	0(0)
统计量		Z＝－2.16	Z＝－0.65	Z＝－0.07	Z＝－0.78	Z＝－0.36	—^b
P值		0.030	0.552	0.972	0.448	0.789	0.170

表3 研究组和对照组LN患儿肾组织病理学特征相关指标比较

组别	例数	LN起病至进行肾组织活检时间[d，M(Q₁，Q₃)]	LN病理学分型[例数(%)]			肾小球坏死[例数(%)]	间质纤维化[例数(%)]	肾小管萎缩[例数(%)]
组别	例数	LN起病至进行肾组织活检时间[d，M(Q₁，Q₃)]	Ⅱ型	Ⅲ型(Ⅲ＋Ⅴ型)	Ⅳ型(Ⅳ＋Ⅴ型)	肾小球坏死[例数(%)]	间质纤维化[例数(%)]	肾小管萎缩[例数(%)]
研究组	9	42.0(32.0，77.0)	0(0)	1(11.1)	8(88.9)	9(100.0)	7(77.8)	4(44.4)
对照组	44	28.0(21.0，65.7)	1(2.3)	4(9.1)	39(88.6)	25(56.8)	24(54.5)	10(22.7)
统计量		Z＝－1.54	—^a			—^b	χ²＝0.84^c	χ²＝0.87^c
P值		0.128	>0.999			0.012	0.359	0.352
组别	例数	细胞/纤维性新月体占比[%，M(Q₁，Q₃)]	细胞/纤维性新月体积分[分，M(Q₁，Q₃)]	肾小管萎缩＋间质纤维化积分[分，M(Q₁，Q₃)]	AI评分[分，M(Q₁，Q₃)]	CI评分[分，M(Q₁，Q₃)]	寡免疫复合物型LN[例数(%)]
研究组	9	26.7(13.0，35.0)	2(0，4)	0(0，0.5)	12.0(7.0，13.0)	0(0，1.5)	1(11.1)
对照组	44	8.0(0，21.5)	2(0，2)	0(0，0)	9.0(7.0，12.0)	0(0，1.0)	0(0)
统计量		Z＝－2.16	Z＝－0.65	Z＝－0.07	Z＝－0.78	Z＝－0.36	—^b
P值		0.030	0.552	0.972	0.448	0.789	0.170

图1 研究组和对照组LN患儿持续CR和生存曲线比较注：研究组为血清ANCA结果呈阳性的LN患儿，对照组为血清ANCA结果呈阴性的LN患儿。图1B中的生存曲线、生存率分别指未进展至ESKD曲线、未进展至ESKD率。ANCA为抗中性粒细胞细胞质抗体，LN为狼疮性肾炎。CR为完全缓解，ESKD为终末期肾病

表4 研究组和对照组LN患儿随访时间、治疗方案及疗效比较

组别	例数	随访时间[月，M(Q₁，Q₃)]	CTX诱导治疗[例数(%)]	联合CNI治疗[例数(%)]	联合HCQ治疗[例数(%)]	治疗6个月达CR[%(n/n′)]	治疗12个月达CR[%(n/n′)]	LN复发[例数(%)]	ESKD[例数(%)]
研究组	9	42.0(21.5，63.5)	7(77.8)	1(11.1)	6(66.7)	88.9(8/9)	88.9(8/9)	3(33.3)	1(11.1)
对照组	44	16.5(6.0，36.8)	36(81.8)	13(29.5)	22(50.0)	80.6(29/36)	86.7(26/30)	8(18.2)	0(0)
统计量		Z＝－1.93	χ²＝1.68^a	χ²＝0.53^a	χ²＝0.30^a	χ²＝0.01^a	—^b	χ²＝0.33^a	—^b
P值		0.052	0.433	0.467	0.585	0.922	1.000	0.569	0.171

表4 研究组和对照组LN患儿随访时间、治疗方案及疗效比较

组别	例数	随访时间[月，M(Q₁，Q₃)]	CTX诱导治疗[例数(%)]	联合CNI治疗[例数(%)]	联合HCQ治疗[例数(%)]	治疗6个月达CR[%(n/n′)]	治疗12个月达CR[%(n/n′)]	LN复发[例数(%)]	ESKD[例数(%)]
研究组	9	42.0(21.5，63.5)	7(77.8)	1(11.1)	6(66.7)	88.9(8/9)	88.9(8/9)	3(33.3)	1(11.1)
对照组	44	16.5(6.0，36.8)	36(81.8)	13(29.5)	22(50.0)	80.6(29/36)	86.7(26/30)	8(18.2)	0(0)
统计量		Z＝－1.93	χ²＝1.68^a	χ²＝0.53^a	χ²＝0.30^a	χ²＝0.01^a	—^b	χ²＝0.33^a	—^b
P值		0.052	0.433	0.467	0.585	0.922	1.000	0.569	0.171

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