Zhu Yingzi, Dong Lingli
Department of Rheumatology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Int J Emerg Med. 2025 Apr 7;18(1):71. doi: 10.1186/s12245-025-00868-6.
Wunderlich syndrome (WS) is a rare condition characterized by spontaneous renal hemorrhage in the absence of obvious trauma or iatrogenic injury. Given that most WS cases are life-threatening and require prompt intervention, timely identification and resolution are essential. Patients with connective tissue diseases (CTDs) account for a small proportion of reported WS cases; however, owing to the specific pathogenic mechanisms and treatments associated with CTDs, these patients exhibit distinctive pathological traits and clinical features in WS.
We present the identification and treatment process of WS in a patient with systemic lupus erythematosus. This patient suffered from sudden abdominal pain and a drastic decline in hemoglobin level accompanied by confusion of consciousness. After the abdominal computerized tomography scan revealed the presence of a renal hematoma, transcatheter arterial embolization was performed on her. Unexpectedly, three days later, the patient had severe anemia and consciousness disorders again. Highly suspecting renal rebleeding, we performed a repeated angiography for the patient. After confirming the bleeding, embolization was carried out again. The renal bleeding stopped, and the patient's hemoglobin level gradually stabilized. Regrettably, this patient ultimately died due to multiple systemic infections.
WS that occurs in CTDs can evolve into critical and severe conditions. Infection, immune complex deposition, thrombocytopenia, abnormal coagulation function, complement activation, autoantibodies production, and glucocorticoid treatment in patients with CTDs are potentially linked to the development of WS. The treatment strategies for WS should be guided by hemodynamic status.
肾自发性出血综合征(WS)是一种罕见病症,其特征为在无明显外伤或医源性损伤的情况下发生自发性肾出血。鉴于大多数WS病例危及生命且需要及时干预,及时识别和解决至关重要。结缔组织病(CTD)患者在已报道的WS病例中占比小;然而,由于与CTD相关的特定致病机制和治疗方法,这些患者在WS中表现出独特的病理特征和临床特点。
我们展示了一名系统性红斑狼疮患者中WS的识别和治疗过程。该患者突发腹痛,血红蛋白水平急剧下降并伴有意识模糊。腹部计算机断层扫描显示存在肾血肿后,对其进行了经导管动脉栓塞术。出乎意料的是,三天后,患者再次出现严重贫血和意识障碍。高度怀疑肾再次出血,我们为患者进行了重复血管造影。确认出血后,再次进行栓塞。肾出血停止,患者的血红蛋白水平逐渐稳定。遗憾的是,该患者最终因多重全身感染死亡。
CTD中发生的WS可发展为危急重症。CTD患者中的感染、免疫复合物沉积、血小板减少、凝血功能异常、补体激活、自身抗体产生以及糖皮质激素治疗可能与WS的发生有关。WS的治疗策略应以血流动力学状态为指导。