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Abstract：
BACKGROUND: IgG4-related disease (IgG4-RD) is a newly discovered systemic disease that can affect any organ or tissue in the body. IgG4-related kidney disease (IgG4-RKD) is relatively rare but essential to IgG4-RD. However, there are few reports of IgG4-RD mimicking malignant ureteral tumors leading to hydronephrosis. We report here a rare case of IgG4-RD involving the ureter.
METHODS: An 87-year-old man presented to our nephrology department with anorexia, nausea, and acute kidney injury in November 2020. Urinary computed tomography (CT) examination revealed a right lower ureter mass with right renal and ureter hydronephrosis. The serum level of IgG4 was 1890 mg/dL, and the concurrently renal biopsy revealed extensive infiltration of IgG4-positive plasma cells in renal interstitium, which was diagnosed as IgG4-associated tubule-interstitial nephritis(IgG4-TIN). The renal function improved significantly after double-J tube implantation of the right ureter and moderate-dose hormone therapy. The serum IgG4 decreased to the normal range, and the right lower ureter mass almost disappeared after one year of low-dose hormone maintenance therapy.
CONCLUSIONS: IgG4-RD can present as a mass in the renal pelvis and (or) ureter, leading to hydronephrosis. Therefore, early recognition of this disease is significant. Most patients respond well to hormonal therapy to avoid surgical treatment due to misdiagnosis as malignant tumors, causing secondary harm to patients.
METHODS: An 87-year-old man presented to our nephrology department with anorexia, nausea, and acute kidney injury in November 2020. Urinary computed tomography (CT) examination revealed a right lower ureter mass with right renal and ureter hydronephrosis. The serum level of IgG4 was 1890 mg/dL, and the concurrently renal biopsy revealed extensive infiltration of IgG4-positive plasma cells in renal interstitium, which was diagnosed as IgG4-associated tubule-interstitial nephritis(IgG4-TIN). The renal function improved significantly after double-J tube implantation of the right ureter and moderate-dose hormone therapy. The serum IgG4 decreased to the normal range, and the right lower ureter mass almost disappeared after one year of low-dose hormone maintenance therapy.
CONCLUSIONS: IgG4-RD can present as a mass in the renal pelvis and (or) ureter, leading to hydronephrosis. Therefore, early recognition of this disease is significant. Most patients respond well to hormonal therapy to avoid surgical treatment due to misdiagnosis as malignant tumors, causing secondary harm to patients.
摘要：
背景：IgG4相关疾病（IgG4-RD）是一种新发现的全身性疾病，可影响体内任何器官或组织。IgG4相关的肾脏疾病(IgG4-RKD)相对罕见，但对IgG4-RD至关重要。然而,IgG4-RD模拟输尿管恶性肿瘤导致肾积水的报道很少.我们在此报告一例罕见的IgG4-RD累及输尿管。
方法：一名87岁的男子因厌食症出现在我们的肾脏科，恶心,以及2020年11月的急性肾损伤。尿液计算机断层扫描（CT）检查显示右下输尿管肿块伴右肾和输尿管肾积水。血清IgG4水平为1890mg/dL,同时肾活检显示肾间质中IgG4阳性浆细胞广泛浸润，被诊断为IgG4相关的小管间质性肾炎（IgG4-TIN）。右输尿管双J管植入和中等剂量激素治疗后肾功能明显改善。血清IgG4降到正常范围,低剂量激素维持治疗一年后，右下输尿管肿块几乎消失。
结论：IgG4-RD可以表现为肾盂和（或）输尿管中的肿块，导致肾积水.因此,对这种疾病的早期识别意义重大。大多数患者对激素治疗反应良好，以避免因误诊为恶性肿瘤而进行手术治疗，对患者造成二次伤害。
方法：一名87岁的男子因厌食症出现在我们的肾脏科，恶心,以及2020年11月的急性肾损伤。尿液计算机断层扫描（CT）检查显示右下输尿管肿块伴右肾和输尿管肾积水。血清IgG4水平为1890mg/dL,同时肾活检显示肾间质中IgG4阳性浆细胞广泛浸润，被诊断为IgG4相关的小管间质性肾炎（IgG4-TIN）。右输尿管双J管植入和中等剂量激素治疗后肾功能明显改善。血清IgG4降到正常范围,低剂量激素维持治疗一年后，右下输尿管肿块几乎消失。
结论：IgG4-RD可以表现为肾盂和（或）输尿管中的肿块，导致肾积水.因此,对这种疾病的早期识别意义重大。大多数患者对激素治疗反应良好，以避免因误诊为恶性肿瘤而进行手术治疗，对患者造成二次伤害。
