Clinical analysis of 4 cases of IgG4-related kidney disease

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

Abstract

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

CLC Number:

R593.2

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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URL: https://huicui.hebmu.edu.cn/EN/10.3969/j.issn.1004-583X.2023.11.010

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

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References 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