PDF(Pubmed)
Abstract：
Phospholipase A2 receptor (PLA2R)-associated membranous nephropathy is an important cause of nephrotic syndrome that can lead to a variety of systemic manifestations. Chylous ascites and bilateral renal vein thrombosis are rare manifestations in adult nephrotic syndrome, and there have been no reported cases demonstrating both chylous ascites and bilateral renal vein thrombosis in patients with PLA2R-associated membranous nephropathy. Here, we report the first case of PLA2R-associated membranous nephropathy complicated by renal vein thrombosis and chylous ascites successfully treated with anticoagulation and rituximab. A 65-year-old African American male presented with abdominal pain for four days, hematochezia for one day, and lower extremity edema for one year. Blood pressure was 158/73 mmHg and other vital signs were normal. Physical examination revealed abdominal distention, periumbilical tenderness, and bilateral lower extremity edema. Laboratory analysis showed high serum creatinine, hypoalbuminemia, hyperlipidemia, and proteinuria on 24-hour urine chemistry, all consistent with nephrotic syndrome. Abdominal computed tomography scan demonstrated nonocclusive bilateral renal vein thrombosis with ascites. Paracentesis revealed chylous ascites. Continuous heparin infusion was started for thrombosis. Esophagoduodenoscopy and colonoscopy did not reveal a source of bleeding. Serum anti-PLA2R was found positive, suggesting membranous nephropathy. Rituximab, along with warfarin switched from heparin, successfully controlled disease activity. Chylous ascites in nephrotic syndrome is thought to be associated with bowel edema. In our case, we hypothesize that renal vein thrombosis caused lymphatic fluid leakage by increasing lymphatic pressure. The case illustrates the importance of considering membranous nephropathy as a cause of chylous ascites and renal vein thrombosis. Development of lymphatic imaging techniques is warranted to clarify the pathophysiology.
摘要：
磷脂酶A2受体(PLA2R)相关性膜性肾病是肾病综合征的重要病因,可导致多种全身表现。乳糜腹水和双侧肾静脉血栓形成是成人肾病综合征的罕见表现。并且在PLA2R相关膜性肾病患者中没有报告同时显示乳糜性腹水和双侧肾静脉血栓形成的病例。这里,我们报道了首例PLA2R相关性膜性肾病并发肾静脉血栓形成和乳糜性腹水的病例,成功应用抗凝和利妥昔单抗治疗.一名65岁的非洲裔美国男性出现腹痛四天，便血一天,下肢水肿一年。血压为158/73mmHg，其他生命体征正常。体格检查显示腹胀，脐周触痛，双侧下肢水肿。实验室分析显示血清肌酐偏高,低蛋白血症,高脂血症,和24小时尿液化学上的蛋白尿，都符合肾病综合征。腹部计算机断层扫描显示非闭塞性双侧肾静脉血栓形成伴腹水。穿刺术显示乳糜腹水。开始持续输注肝素治疗血栓形成。食管十二指肠镜检查和结肠镜检查未发现出血源。血清抗PLA2R阳性，提示膜性肾病.利妥昔单抗,随着华法林从肝素转换过来，成功控制疾病活动。肾病综合征的乳糜腹水被认为与肠水肿有关。在我们的案例中,我们假设肾静脉血栓形成通过增加淋巴压力引起淋巴液渗漏。该案例说明了将膜性肾病视为乳糜性腹水和肾静脉血栓形成的原因的重要性。淋巴成像技术的发展对于阐明病理生理学是必要的。
