温德利希综合征作为结节性多动脉炎的一种表现。
Wunderlich's syndrome as a manifestation of polyarteritis nodosa.
作者信息
Beirão Pedro, Teixeira Lídia, Pereira Patrícia, Coelho Margarida Lopes
机构信息
Department of Internal Medicine, Hospital Garcia de Orta, Almada, Portugal.
Department of Rheumatology, Hospital Garcia de Orta, Almada, Portugal.
出版信息
BMJ Case Rep. 2017 Jan 30;2017:bcr2016218478. doi: 10.1136/bcr-2016-218478.
Wunderlich syndrome is a rare clinical entity characterised by spontaneous renal haemorrhage that can be life-threatening and requires emergency attention. The most frequent aetiologies are renal tumours and vascular diseases. The authors report a case of a patient admitted with hypovolaemic shock due to a retroperitoneal haematoma in the context of polyarteritis nodosa (PAN). Prognosis of untreated PAN is very poor with 13% 5-year survival rate. Therefore, early recognition of disease and proper treatment with immunosuppressors will prevent catastrophic complications and improves survival.
温德利希综合征是一种罕见的临床病症，其特征为自发性肾出血，可能危及生命，需要紧急处理。最常见的病因是肾肿瘤和血管疾病。作者报告了一例结节性多动脉炎（PAN）患者因腹膜后血肿导致低血容量性休克入院的病例。未经治疗的PAN预后很差，5年生存率为13%。因此，早期识别疾病并使用免疫抑制剂进行适当治疗将预防灾难性并发症并提高生存率。
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