Naito Shotaro, Yamaguchi Hiroyuki, Hagino Noboru
Department of General Internal Medicine, Fukushima Medical University, Fukushima, JPN.
Department of Rheumatology, Teikyo University Chiba Medical Center, Chiba, JPN.
Cureus. 2023 Aug 2;15(8):e42837. doi: 10.7759/cureus.42837. eCollection 2023 Aug.
Systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare and potentially lethal condition characterized by hypotension, hemoconcentration, and hypoalbuminemia; however, the cause of SCLS is still uncertain. We present the case of a 62-year-old male with flu-like symptoms who presented to the emergency department with shock. Initial evaluation revealed hemoconcentration, hypoalbuminemia, acute kidney failure, and positive polymerase chain reaction (PCR) for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Despite aggressive fluid resuscitation, the shock persisted, and the patient's condition deteriorated. After ruling out ischemia and septic shock, the patient was diagnosed with coronavirus disease 2019 (COVID-19)-associated SCLS. Treatment with remdesivir and intravenous immunoglobulin (IVIG), along with the restoration of intravascular volume, led to the gradual improvement of the patient's condition. The patient experienced pulmonary edema, which was managed by correcting the fluid balance through continuous hemodiafiltration. Eventually, the patient recovered without any residual organ complications. SCLS is often misdiagnosed because of its rarity and non-specific symptoms. Accurate diagnosis and understanding of the disease's pathophysiology are crucial for effective management. This report contributes to the existing literature by presenting a case of COVID-19-associated SCLS and emphasizes the need for further research on its occurrence and outcomes.
系统性毛细血管渗漏综合征(SCLS),也称为克拉克森病,是一种罕见且可能致命的疾病,其特征为低血压、血液浓缩和低白蛋白血症;然而,SCLS的病因仍不明确。我们报告一例62岁男性病例,该患者有流感样症状,因休克就诊于急诊科。初始评估显示血液浓缩、低白蛋白血症、急性肾衰竭以及严重急性呼吸综合征冠状病毒2(SARS-CoV-2)的聚合酶链反应(PCR)呈阳性。尽管进行了积极的液体复苏,休克仍持续存在,患者病情恶化。在排除缺血性休克和感染性休克后,该患者被诊断为2019冠状病毒病(COVID-19)相关的SCLS。使用瑞德西韦和静脉注射免疫球蛋白(IVIG)治疗,同时补充血管内容量,使患者病情逐渐好转。患者出现了肺水肿,通过持续血液透析滤过纠正液体平衡进行处理。最终,患者康复,未遗留任何器官并发症。由于SCLS罕见且症状不具特异性,常被误诊。准确诊断并了解该疾病的病理生理学对于有效治疗至关重要。本报告通过呈现一例COVID-19相关的SCLS病例,为现有文献做出了贡献,并强调了对其发生情况和转归进行进一步研究的必要性。