IgG4相关性肾病4例临床分析

doi:10.3969/j.issn.1004-583X.2023.11.010

摘要/Abstract

摘要：

目的探讨IgG4相关性肾病的临床表现、实验室及影像学特点、肾脏病理特征和治疗方案。方法回顾性分析2016年3月至 2021年3月于广东省中医院珠海医院肾病科住院经肾穿刺活检确诊为IgG4 相关性肾病的患者4例,比较其临床表现、实验室及影像学特点、肾脏病理特征及治疗效果。结果共纳入患者4例,其中男性3例,女性1例,中位年龄71岁;急性起病2例,慢性病程2例;病变累及肾脏和胰腺1例,病变累及肾脏和腹膜后1例;主要临床表现多样,无特异性;4例均出现血清肌酐升高伴少量蛋白尿,血清IgG4显著升高3例;4例均经肾脏病理诊断符合IgG4 相关性肾小管-间质肾炎;4例均接受糖皮质激素治疗,疗效明显。结论 IgG4相关性肾病临床罕见,易造成漏诊误诊,血清IgG4筛查有助于提高诊断率,肾穿刺活检是诊断的金标准,糖皮质激素治疗有效,随访效果较好。

Abstract:

Objective To explore the clinical manifestations, laboratory and imaging characteristics, pathological features of the kidney and treatment regimen for immunoglobulin G4 (IgG4)-related kidney disease (IgG4-RKD). Methods Four cases of IgG4-RKD diagnosed by renal biopsy in Department of Nephrology, Guangdong Provincial Hospital of Chinese Medicine, Zhuhai from March 2016 to March 2021 were retrospectively analyzed. Their clinical manifestations, laboratory and imaging characteristics, pathological features of the kidney and therapeutic outcome were compared. Results A total of 4 cases of IgG-RKD were included, involving three males and one female with the median age of 71 years. Two cases presented acute onset and two cases were chronic course. The injured organs of one case were kidney and pancreas and the other case was affected by the kidney and retroperitoneum. The main clinical manifestations were varied and unspecific. Four cases developed elevated serum creatinine with mild proteinuria. Three cases presented significantly increased serum IgG4. All patients were diagnosed with IgG4-related tubulointerstitial nephritis. Patients received glucocorticoid therapy with a significant therapeutic efficacy. Conclusion IgG4-RKD is rare and prone to missed diagnosis and misdiagnosis. Serum IgG4 screening can help to elevate the diagnosis rate. Renal biopsy is the gold standard for diagnosing IgG4-RKD. Glucocorticoid therapy is effective and obtains a favorable follow-up outcome.

Key words: immunoglobulin G4-related disease, clinical manifestation, immunoglobulin G, renal pathology, glucocorticoids

中图分类号:

R593.2

何培华, 周幸福, 洪炜鸿, 王利纯, 刘素君, 金玉燕, 曾佳豪, 刘立昌. IgG4相关性肾病4例临床分析[J]. 临床荟萃, 2023, 38(11): 1016-1021.

He Peihua, Zhou Xingfu, Hong Weihong, Wang Lichun, Liu Sujun, Jin Yuyan, Zeng Jiahao, Liu Lichang. Clinical analysis of 4 cases of IgG4-related kidney disease[J]. Clinical Focus, 2023, 38(11): 1016-1021.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2023.11.010

https://huicui.hebmu.edu.cn/CN/Y2023/V38/I11/1016

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参考文献 33

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病例	年龄(岁)	性别	病程(月)	主要症状	体格检查	受累器官
患者1	76	男	1	乏力、纳差、少尿至无尿	左下肢轻度凹陷性浮肿	肾脏
患者2	66	女	1	乏力、纳差	无特殊	肾脏
患者3	88	男	48	乏力、纳差、腹痛	双足背轻度凹陷性浮肿	肾脏、胰腺
患者4	56	男	9	乏力、纳差、恶心呕吐	左下肢轻度凹陷性浮肿	肾脏、腹膜后

病例	年龄(岁)	性别	病程(月)	主要症状	体格检查	受累器官
患者1	76	男	1	乏力、纳差、少尿至无尿	左下肢轻度凹陷性浮肿	肾脏
患者2	66	女	1	乏力、纳差	无特殊	肾脏
患者3	88	男	48	乏力、纳差、腹痛	双足背轻度凹陷性浮肿	肾脏、胰腺
患者4	56	男	9	乏力、纳差、恶心呕吐	左下肢轻度凹陷性浮肿	肾脏、腹膜后

病例	血清肌酐 (58.0~110.0 μmol/L)		血清IgG (8.00~17.00 g/L)		血清IgG4 (0.08~1.35 g/L)		补体3 (0.90~1.80 g/L)		补体4 (0.10~0.40 g/L)		尿微量白蛋白 (0.0~20.0 mg/L)
患者1	1 503.1		16.96		NA		0.63		0.16		146.4
患者2	271.0		23.1		3.09		1.52		0.48		NA
患者3	129.1		23.03		3.20		1.67		0.41		19.0
患者4	1 448.6		22.42		3.00		0.40		0.07		59.6
患者	尿β2微球蛋白 (0.11~0.32 mg/L)	24h尿蛋白总量 (mg/24h) (<150 mg/24h)		血清白蛋白 (40.0~55.0 g/L)		血红蛋白 (130~175 g/L)		血清淀粉酶 (35.0~135.0 U/L)		血清脂肪酶 (23.0~300.0 U/L)
患者1	7.79	NA		30.3		85		84.0		NA
患者2	NA	1277.65		39.5		108		NA		NA
患者3	0.55	191.31		31.0		99		316.0		1274.5
患者4	10.30	1400.64		35.8		96		172.7		478.2

病例	血清肌酐 (58.0~110.0 μmol/L)		血清IgG (8.00~17.00 g/L)		血清IgG4 (0.08~1.35 g/L)		补体3 (0.90~1.80 g/L)		补体4 (0.10~0.40 g/L)		尿微量白蛋白 (0.0~20.0 mg/L)
患者1	1 503.1		16.96		NA		0.63		0.16		146.4
患者2	271.0		23.1		3.09		1.52		0.48		NA
患者3	129.1		23.03		3.20		1.67		0.41		19.0
患者4	1 448.6		22.42		3.00		0.40		0.07		59.6
患者	尿β2微球蛋白 (0.11~0.32 mg/L)	24h尿蛋白总量 (mg/24h) (<150 mg/24h)		血清白蛋白 (40.0~55.0 g/L)		血红蛋白 (130~175 g/L)		血清淀粉酶 (35.0~135.0 U/L)		血清脂肪酶 (23.0~300.0 U/L)
患者1	7.79	NA		30.3		85		84.0		NA
患者2	NA	1277.65		39.5		108		NA		NA
患者3	0.55	191.31		31.0		99		316.0		1274.5
患者4	10.30	1400.64		35.8		96		172.7		478.2

肾脏病理	患者1	患者2	患者3	患者4
光镜
肾小球总数(个)	5	20	10	3
肾小球病变(个)	0	0	2	1
肾间质病变
肾间质纤维化	灶状	灶状	鸟眼状	灶状
炎症细胞浸润	弥漫性	弥漫性	弥漫性	弥漫性
肾小管萎缩	部分	无	灶状(萎缩面积>30%)	灶状(萎缩面积>10%)
免疫荧光	IgM+	IgM+	IgM+	无
免疫组化	部分浆细胞IgG4染色阳性	IgG4/IgG<40%,IgG4阳性细胞数局灶区域>10/HPF	IgG4/IgG<40%,IgG4阳性细胞数局灶区域>10/HPF	IgG4/IgG>40%,IgG4阳性细胞数局灶区域>10/HPF
电镜
电子致密物沉积	无	无	无	无
诊断	IgG4-TIN	IgG4-TIN	IgG4-TIN	IgG4-TIN