Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan.
Am J Kidney Dis. 2013 Jan;61(1):167-70. doi: 10.1053/j.ajkd.2012.06.027. Epub 2012 Sep 14.
Spontaneous perinephric hemorrhage is a clinically rare life-threatening condition, also known as Wünderlich syndrome. Presentations can be variable, but patients typically display symptoms and signs including flank or abdominal pain, abdominal mass, and hypovolemia. It is important to diagnose the syndrome early because untreated cases carry a high mortality risk, and prompt intervention with an endovascular procedure or surgery is life saving. Causes range from anatomical anomalies, including vascular diseases (vasculitides and aneurysms) and renal tumors, to functional coagulation defect (bleeding tendency). The most common causes of Wünderlich syndrome are renal angiomyolipoma and renal cell carcinoma, constituting 60%~70% of cases. Vascular causes of Wünderlich syndrome are infrequent, and the culprit most frequently is vasculitis resulting from polyarteritis nodosa. Other vasculitides presenting as Wünderlich syndrome are infrequent. We describe a 39-year-old woman with end-stage renal disease from lupus nephritis and spontaneous renal hemorrhage, ascribed to lupus-related vasculitis after serologic testing, computed tomography, and angiographic studies.
自发性肾周出血是一种临床罕见的危及生命的疾病，也称为 Wünderlich 综合征。临床表现多样，但患者通常表现出腰痛或腹痛、腹部肿块和低血容量等症状和体征。早期诊断该综合征非常重要，因为未经治疗的病例死亡率很高，及时进行血管内介入治疗或手术可以挽救生命。其病因包括从解剖异常（血管疾病[血管炎和动脉瘤]和肾肿瘤]）到功能性凝血缺陷（出血倾向）。Wünderlich 综合征最常见的病因是肾血管平滑肌脂肪瘤和肾细胞癌，占病例的 60%~70%。血管性 Wünderlich 综合征并不常见，最常见的病因是结节性多动脉炎引起的血管炎。其他以 Wünderlich 综合征为表现的血管炎则较为罕见。我们描述了一例 39 岁女性，患有狼疮肾炎导致的终末期肾病和自发性肾出血，血清学检查、计算机断层扫描和血管造影研究后归因于狼疮相关血管炎。
