全身型幼年特发性关节炎合并肾梗死患儿的临床特征、诊疗及预后

doi:10.3877/cma.j.issn.1673-5250.2026.04.007

目的

探讨全身型幼年特发性关节炎(SJIA)合并肾梗死患儿的临床特征、诊疗及预后。

方法

选择2014年7月至2025年5月于首都医科大学附属首都儿童医学中心风湿免疫科诊治的3例SJIA合并肾梗死患儿为研究对象。采用回顾性分析方法，对其临床病例资料，包括临床特征、实验室检查结果、影像学特征、治疗经过及随访结果等进行研究。同时以"幼年特发性关节炎""肾梗死""关节炎""renal infarction""juvenile idiopathic arthritis""arthritis"等为中、英文关键词，检索国内外数据库中关于SJIA或关节炎合并肾梗死的文献，并进行综合分析。本研究遵循的程序符合首都医科大学附属首都儿童医学中心制定的伦理学标准，并获得该伦理委员会批准(审批文号：SHERLLM2021046)。

结果

①3例患儿中，女性为2例、男性为1例，年龄分别为7、6、15岁。3例患儿均处于SJIA活动期，临床特征均为反复发热、皮疹及关节炎，并伴不同程度多系统受累。实验室检查结果提示，3例患儿均存在明显炎症反应，表现为白细胞计数(WBC)、C反应蛋白(CRP)、红细胞沉降率(ESR)及血清铁蛋白(SF)水平升高；同时均存在凝血及纤溶系统异常，表现为D-二聚体(D-D)及纤维蛋白降解产物(FDP)水平升高。3例患儿中，2例有腹痛(1例伴腰痛及肾区叩击痛，1例伴血尿和蛋白尿；1例无明显腹部症状)。腹部增强CT结果均显示，肾脏楔形低密度或低强化灌注缺损区，其中右肾受累为1例、左肾受累为1例、双肾受累为1例。这3例SJIA合并肾梗死患儿早期，均曾考虑为肾脓肿可能，采取抗菌药物治疗后，上述临床症状及肾脏病灶无明显改善；经积极控制SJIA活动度，并联合抗凝治疗，对部分患儿加用抗血小板治疗后，患儿发热、腹痛及关节炎症状缓解。随访期间，3例患儿肾脏梗死病灶均较前缩小或吸收，肾功能均未见异常。②文献复习结果显示，采用本研究设定的检索策略，未检索到SJIA合并肾梗死患儿的相关文献报道；进一步扩大至风湿免疫性疾病相关关节炎后，仅检索到1例成年类风湿关节炎合并肾梗死患者的病例报道。这例患者的血管CT检查结果显示多支动脉血管炎性改变，最终考虑为类风湿血管炎所致肾梗死。

结论

SJIA合并肾梗死临床罕见，患儿的临床特征缺乏特异性，容易被误诊或漏诊。肾梗死可能是SJIA活动期的并发症。腹部增强CT有助于SJIA合并肾梗死患儿的早期诊断。及时控制SJIA病情活动度，并结合凝血状态给予抗凝或抗血小板治疗，可能有助于改善SJIA合并肾梗死患儿肾脏转归。

关键词: 幼年特发性关节炎全身型, 肾梗死, 糖皮质激素, 关节炎，类风湿, 关节炎，青少年, 体层摄影术，X线计算机, 历史文献, 儿童

Objective

To investigate the clinical characteristics, diagnosis, treatment, and prognosis of renal infarction in children with systemic juvenile idiopathic arthritis (SJIA).

Methods

Three children with SJIA complicated by renal infarction who were diagnosed and treated in the Department of Rheumatology and Immunology, Capital Center for Children′s Health Capital Medical University, from July 2014 to May 2025 were selected into this study. Their clinical data were retrospectively analyzed, including clinical manifestations, laboratory findings, imaging features, treatment, and follow-up outcomes. In addition, Chinese and English databases were searched using " juvenile idiopathic arthritis" " renal infarction", and " arthritis" as search terms to identify studies on SJIA or arthritis complicated by renal infarction, and the retrieved literature was comprehensively analyzed. The study protocol was conducted in accordance with the ethical standards of Capital Center for Children′s Health Capital Medical University, and was approved by its Ethics Committee (Approval No. SHERLLM2021046).

Results

① Among the 3 children, 2 were female and 1 was male, aged 7, 6, and 15 years, respectively. All 3 children were in the active phase of SJIA, presenting with recurrent fever, rash, and arthritis, accompanied by multisystem involvement. Laboratory tests showed marked inflammatory responses in all 3 children, as indicated by elevated white blood cell count (WBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and serum ferritin (SF) levels. Abnormalities of the coagulation and fibrinolytic systems were also observed in all 3 children, manifested by increased D-dimer (D-D) and fibrin degradation product (FDP) levels. Among the 3 children, 2 had abdominal pain, of whom 1 also had low back pain and renal percussion tenderness, and 1 had hematuria and proteinuria; the remaining child had no obvious abdominal symptoms. Contrast-enhanced abdominal CT showed wedge-shaped low-density areas or hypoenhancing perfusion defects in the kidney in all 3 children, with right kidney involvement in 1 case, left in 1 case, and bilateral in 1 case. Renal abscess was initially considered in all 3 children. However, after antimicrobial therapy, the above clinical symptoms and renal lesions showed no obvious improvement. After active control of SJIA activity combined with anticoagulant therapy, with antiplatelet therapy added in some cases, fever, abdominal pain, and joint symptoms were relieved. During follow-up, renal lesions decreased in size or resolved in all 3 children, and no renal dysfunction was observed. The literature review identified no published reports of renal infarction in children with SJIA. After expanding the search to arthritis-related rheumatic diseases, only one adult case of rheumatoid arthritis complicated by renal infarction was identified.

Conclusions

Renal infarction in children with SJIA is rare and has nonspecific clinical manifestations, which may lead to misdiagnosis or delayed diagnosis. Renal infarction may represent a complication during the active phase of SJIA. Contrast-enhanced abdominal CT is useful for early diagnosis. Timely control of SJIA disease activity, together with anticoagulant or antiplatelet therapy based on coagulation status, may help improve renal outcomes.

Key words: Systemic juvenile idiopathic arthritis, Renal infarction, Glucocorticoids, Arthritis, rheumatoid, Arthritis, juvenile, Tomography, X-ray computed

表1 SJIA合并肾梗死患儿1~3本次入院的一般临床资料比较

患儿编号	年龄(岁)/性别	病程(d)^a	发热/皮疹/关节炎	腹痛/腰痛	双侧肾区叩击痛	血尿/蛋白尿	其他器官受累
患儿1	7/女性	15	有/有/有	有/有	有	无/无	血液系统、浆膜腔、心脏
患儿2	6/女性	14	有/有/有	有/无	无	有/有	血液系统、浆膜腔、心脏
患儿3	15/男性	29	有/有/有	无/无	无	无/无	血液系统、浆膜腔、心脏、淋巴结

表1 SJIA合并肾梗死患儿1~3本次入院的一般临床资料比较

患儿编号	年龄(岁)/性别	病程(d)^a	发热/皮疹/关节炎	腹痛/腰痛	双侧肾区叩击痛	血尿/蛋白尿	其他器官受累
患儿1	7/女性	15	有/有/有	有/有	有	无/无	血液系统、浆膜腔、心脏
患儿2	6/女性	14	有/有/有	有/无	无	有/有	血液系统、浆膜腔、心脏
患儿3	15/男性	29	有/有/有	无/无	无	无/无	血液系统、浆膜腔、心脏、淋巴结

表2 本研究SJIA合并肾梗死患儿1~3本次入院实验室检查结果比较

患儿编号	WBC(×10⁹/L)	Hb(g/L)	PLT(×10⁹/L)	CRP(mg/L)	ESR(mm/h)	SF(ng/mL)	AST(U/L)	ALT(U/L)
患儿1	26.6	52	877	170.0	119	2 000	45	33
患儿2	41.2	87	421	227.1	90	31 058	45	47
患儿3	24.7	122	256	21.9	64	18 044	225	494
正常参考值	4~11	120~160	100~300	<8	0~20	10~120	0~40	0~40
患儿编号	LDH(U/L)	TG(mmol/L)	D-D	FDP(μg/mL)	FIB(g/L)	病原学(＋/－)
患儿1	495	1.76	>1 050 μg/L^a	30.5	7.0	支原体可疑(＋)
患儿2	579	1.20	15.06 mg/LFEU^b	29.7	6.9	副流感病毒(＋)、人偏肺病毒(＋)
患儿3	766	1.44	11.34 mg/LFEU^b	24.5	5.0	腺病毒(＋)
正常参考值	80~300	0~1.69	—^a，b	<5	2~4	(－)

表2 本研究SJIA合并肾梗死患儿1~3本次入院实验室检查结果比较

患儿编号	WBC(×10⁹/L)	Hb(g/L)	PLT(×10⁹/L)	CRP(mg/L)	ESR(mm/h)	SF(ng/mL)	AST(U/L)	ALT(U/L)
患儿1	26.6	52	877	170.0	119	2 000	45	33
患儿2	41.2	87	421	227.1	90	31 058	45	47
患儿3	24.7	122	256	21.9	64	18 044	225	494
正常参考值	4~11	120~160	100~300	<8	0~20	10~120	0~40	0~40
患儿编号	LDH(U/L)	TG(mmol/L)	D-D	FDP(μg/mL)	FIB(g/L)	病原学(＋/－)
患儿1	495	1.76	>1 050 μg/L^a	30.5	7.0	支原体可疑(＋)
患儿2	579	1.20	15.06 mg/LFEU^b	29.7	6.9	副流感病毒(＋)、人偏肺病毒(＋)
患儿3	766	1.44	11.34 mg/LFEU^b	24.5	5.0	腺病毒(＋)
正常参考值	80~300	0~1.69	—^a，b	<5	2~4	(－)

图1 患儿1(女性，7岁)治疗前、后腹部增强CT影像图[图1A：治疗前，可见右侧肾脏下极于增强后见楔形低密度区(红色箭头所示)，肾脏大小正常；图1B：治疗后，可见右肾楔形低密度区消失(红色箭头所示)]注：患儿1为SJIA合并肾梗死患儿。SJIA为全身型幼年特发性关节炎

表3 本研究SJIA合并肾梗死患儿本次入院的治疗方案及转归比较

患儿编号	MPPT [mg/(kg·d)]	常规剂量甲泼尼龙[mg/(kg·d)]	环孢素[mg/(kg·d)]	甲氨蝶呤[mg/(m²·7 d)]	生物制剂	低分子肝素/阿司匹林	其他药物	转归^c
患儿1	20	1.5	6.5	10	无	有/无	抗菌药物、塞来昔布	病程第35天病灶消失
患儿2	15	1.0	3.0	10	托珠单抗	有/有	抗菌药物、人免疫球蛋白	病程第20个月病灶消失
患儿3	0^a	0.8	3.0	无	无^b	有/无	抗菌药物、双氯芬酸钠、芦可替尼	病程第72天病灶缩小

表3 本研究SJIA合并肾梗死患儿本次入院的治疗方案及转归比较

患儿编号	MPPT [mg/(kg·d)]	常规剂量甲泼尼龙[mg/(kg·d)]	环孢素[mg/(kg·d)]	甲氨蝶呤[mg/(m²·7 d)]	生物制剂	低分子肝素/阿司匹林	其他药物	转归^c
患儿1	20	1.5	6.5	10	无	有/无	抗菌药物、塞来昔布	病程第35天病灶消失
患儿2	15	1.0	3.0	10	托珠单抗	有/有	抗菌药物、人免疫球蛋白	病程第20个月病灶消失
患儿3	0^a	0.8	3.0	无	无^b	有/无	抗菌药物、双氯芬酸钠、芦可替尼	病程第72天病灶缩小

图4 患儿3(男性，15岁)治疗前、后腹部增强CT影像图[图4A：治疗前，腹部增强CT检查结果显示双肾上极类楔形低密度灌注缺损区，边界清晰，右侧病灶大小约为1.2 cm ×1.1 cm ×1.1 cm，左侧约为0.9 cm×0.8 cm×0.7 cm(红色箭头所示)；图4B：治疗后，腹部增强CT检查结果显示，双肾上极楔形低密度区范围缩小，右侧肾梗死病灶大小约为1.1 cm×1.0 cm×1.0 cm，左侧约为0.7 cm×0.6 cm×0.6 cm(红色箭头所示)，密度减低征象改善]

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Clinical characteristics, treatment and prognosis of renal infarction in children with systemic juvenile idiopathic arthritis

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Clinical characteristics, treatment and prognosis of renal infarction in children with systemic juvenile idiopathic arthritis