IgG4相关泌尿系疾病4例报告并文献复习

Report of 4 cases of IgG4-related urinary diseases and literature review

摘要

目的 总结人免疫球蛋白G4（IgG4）相关泌尿系疾病的临床特征，为该疾病的诊断和治疗提供参考。方法 回顾性分析2019年8月-2023年12月于空军军医大学西京医院首诊并经活检病理确诊为IgG4相关泌尿系疾病的4例患者资料，报告患者的诊治过程，对其症状、血清学、影像学和病理学资料以及治疗和转归进行分析。结果 4例患者中男性、女性各2例，病变均主要位于腹膜后，累及泌尿系器官，其中3例有腰痛症状。影像学检查示患者1、3表现为腹膜后占位累及泌尿系器官，患者2主要表现为右肾多发团片状致密影，患者4表现为单纯肾脏囊性占位。4例患者术前未检测血清IgG4值，均行手术根治治疗，术后病理学可见纤维组织增生伴大量浆细胞、淋巴细胞及少许中性粒细胞浸润，部分标本可见淋巴滤泡以及闭塞性血管炎，高倍镜视野下可见组织IgG4⁺浆细胞数量均＞10个。术后3例患者症状得到好转，血清IgG4值均处于正常参考值范围内；余1例（患者3）随访过程中发现血清IgG4升高，于免疫科接受激素治疗，目前血清IgG4水平维持稳定。结论 IgG4相关性疾病累及泌尿系统的症状无特异性，影像学表现多样，易与其他疾病混淆，尽早进行血清IgG4检测、穿刺活检病理可以帮助临床医师早期做出正确诊断。

Abstract

Objective To explore the clinical features of IgG4-related urinary diseases so as to provide reference for the diagnosis and treatment of such diseases. Methods The clinical data of 4 cases of IgG4-related urinary system diseases diagnosed and treated in Xijing Hospital of Air Force Medical University during Aug. 2019 and Dec. 2023 were retrospectively collected. Here, we report on the diagnosis and treatment of these patients, analysing their symptoms, serology, imaging and pathology as well as their treatment and outcomes. Results The patients included 2 male and 2 female. The lesions were involved with the retroperitoneum and urinary system. Three patients had symptoms of lumbar pain. The imaging manifestations were complex, including retroperitoneal mass involving urinary system organs in 2 cases, tabdense shadow of the right kidney in 1 case, and simple cystic mass of kidney in 1 case. Serum IgG4 value was not detected before surgery. All patients underwent radical surgical treatment. Postoperative pathology showed fibrous tissue hyperplasia with a large number of plasma cells, lymphocytes, a few neutrophil infiltrates, and lymphoid follicles and obliterated vasculitis in some specimens. The number of IgG4⁺ plasma cells was more than 10 in all tissues under high power microscope. After surgery, 3 patients had symptoms improved, and serum IgG4 value was within the normal range; 1 patient (patient 3) had elevated IgG4 value during follow-up, received subsequent hormone therapy, and the serum IgG4 level remained stable. Conclusion The symptoms of IgG4-related diseases involving the urinary system are non-specific, and the imaging findings are various, easily confused with other diseases. Early detection of serum IgG4 and biopsy pathology can help clinicians make correct diagnosis in the early stage.

关键词

泌尿系统 / IgG4相关性疾病 / 临床特征 / 影像学表现 / 肾脏疾病

Key words

urinary system / IgG4-related disease / clinical features / imaging manifestations / kidney disease

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