Department of Nephrology, Shinonoi General Hospital, Nagano, Japan.
Department of Nephrology, Shinshu University Hospital, Matsumoto, Japan.
J Int Med Res. 2024 Nov;52(11):3000605241301863. doi: 10.1177/03000605241301863.
Systemic capillary leak syndrome (SCLS) is a rare and life-threatening disorder. A man in his 60s presented for emergency care because of fatigue, decreased urine output and difficulty in moving his body. On admission, he was conscious, afebrile and had relative hypotension. Blood tests and urinary analysis revealed the following: white blood cell count, 19 500/μl; haematocrit, 64.5%; creatinine, 2.16 mg/dl; albumin, 3.3 g/dl; and 0.2% for fractional excretion of sodium. The patient was diagnosed with prerenal acute kidney injury (AKI) and was treated with intravenous fluid administration of more than 2 l/day. His kidney function gradually recovered after 4 days and creatinine decreased (1.15 mg/dl). However, he developed two more attacks of prerenal AKI during hospitalization, one of which needed intensive care unit management. Specific findings of hypotension, haemoconcentration, and hypoalbuminaemia were observed during all AKI attacks. Finally, he was diagnosed as idiopathic SCLS and was treated with intravenous immunoglobulin. SCLS might remain undiagnosed because of its rarity, but it can rapidly progress and lead to severe complications in absence of treatment. Clinicians need to consider this disease as a differential diagnosis when encountering patients who present with frequent prerenal AKI attacks accompanied by hypotension, haemoconcentration and hypoalbuminaemia.
全身性毛细血管渗漏综合征（SCLS）是一种罕见且危及生命的疾病。一位 60 多岁的男性因疲劳、尿量减少和身体活动困难而到急诊就诊。入院时，他神志清醒，无发热，血压相对较低。血液检查和尿液分析显示：白细胞计数 19500/μl；血细胞比容 64.5%；肌酐 2.16mg/dl；白蛋白 3.3g/dl；钠排泄分数 0.2%。患者被诊断为肾前性急性肾损伤（AKI），并接受了每天超过 2 升的静脉补液治疗。他的肾功能在 4 天后逐渐恢复，肌酐下降（1.15mg/dl）。然而，在住院期间他又发生了两次肾前性 AKI 发作，其中一次需要重症监护病房管理。在所有 AKI 发作期间均观察到低血压、血液浓缩和低白蛋白血症的具体表现。最后，他被诊断为特发性 SCLS，并接受了静脉注射免疫球蛋白治疗。由于其罕见性，SCLS 可能未被诊断，但如果不治疗，它可能会迅速进展并导致严重并发症。当遇到频繁发生肾前性 AKI 发作伴有低血压、血液浓缩和低白蛋白血症的患者时，临床医生需要考虑将这种疾病作为鉴别诊断。
