Abstract：
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease characterized by recurrent episodes of acute life-threatening attacks of shock, hemoconcentration, and hypoalbuminemia. Increase in capillary permeability results in reversible plasma movement into the interstitial spaces followed by appearance of related symptoms or complications, including renal failure. This condition can be potentially life-threatening; however, it is easily misdiagnosed.
A 47-year-old man with no previous medical history presented to the emergency department after experiencing general weakness and abdominal pain. He developed hypovolemic shock within 3 h of presentation and initial laboratory tests showed hemoconcentration, hypoalbuminemia and acute kidney injury. Following vigorous fluid therapy and supportive care, the patient recovered, but a similar episode recurred after 4 months without any specific trigger. Based on the combined clinical manifestations and laboratory findings of both the attacks, he was diagnosed with ISCLS. Symptomatic relief was achieved via oxygen supplementation and massive volume replacement using normal saline and the patient was prescribed bambuterol 10 mg and theophylline 400 mg once-a-day. He was discharged from the hospital on day 5 of hospitalization. Thereafter, the patient has been followed for 5 years without any symptoms or recurrence of ISCLS even in the situation of COVID-19 infection.
ISCLS is an extremely infrequent and commonly misdiagnosed disease. However, early diagnosis, treatment and prophylaxis through accumulated clinical data can prevent ISCLS recurrence and the development of related fatal complications. Therefore, clinicians need to be well aware of the variety of clinical characteristics and treatment options of this disease.
A 47-year-old man with no previous medical history presented to the emergency department after experiencing general weakness and abdominal pain. He developed hypovolemic shock within 3 h of presentation and initial laboratory tests showed hemoconcentration, hypoalbuminemia and acute kidney injury. Following vigorous fluid therapy and supportive care, the patient recovered, but a similar episode recurred after 4 months without any specific trigger. Based on the combined clinical manifestations and laboratory findings of both the attacks, he was diagnosed with ISCLS. Symptomatic relief was achieved via oxygen supplementation and massive volume replacement using normal saline and the patient was prescribed bambuterol 10 mg and theophylline 400 mg once-a-day. He was discharged from the hospital on day 5 of hospitalization. Thereafter, the patient has been followed for 5 years without any symptoms or recurrence of ISCLS even in the situation of COVID-19 infection.
ISCLS is an extremely infrequent and commonly misdiagnosed disease. However, early diagnosis, treatment and prophylaxis through accumulated clinical data can prevent ISCLS recurrence and the development of related fatal complications. Therefore, clinicians need to be well aware of the variety of clinical characteristics and treatment options of this disease.
摘要：
背景：特发性系统性毛细血管渗漏综合征（ISCLS）是一种罕见的疾病，其特征是反复发作的急性危及生命的休克发作，血液浓缩,和低蛋白血症.毛细血管通透性的增加导致血浆可逆性移动到间质间隙，随后出现相关症状或并发症。包括肾功能衰竭.这种情况可能危及生命；然而，很容易误诊。
方法：一名没有既往病史的47岁男子在经历全身无力和腹痛后到急诊科就诊。他在就诊后3小时内出现低血容量性休克，最初的实验室检查显示血液浓缩，低蛋白血症和急性肾损伤。经过剧烈的液体治疗和支持治疗，病人康复了,但类似的发作在4个月后复发，没有任何特定的触发因素。根据两次发作的临床表现和实验室检查结果，他被诊断为ISCLS。通过补充氧气和使用生理盐水进行大量置换可缓解症状，并每天一次给患者开10mg班布特罗和400mg茶碱。他在住院第5天出院。此后,即使在COVID-19感染的情况下，患者也已随访5年，没有任何ISCLS症状或复发。
结论：ISCLS是一种极罕见且经常被误诊的疾病。然而,早期诊断,通过积累的临床数据进行治疗和预防可以预防ISCLS复发和相关致命并发症的发展。因此,临床医生需要充分了解本病的各种临床特征和治疗方案.
方法：一名没有既往病史的47岁男子在经历全身无力和腹痛后到急诊科就诊。他在就诊后3小时内出现低血容量性休克，最初的实验室检查显示血液浓缩，低蛋白血症和急性肾损伤。经过剧烈的液体治疗和支持治疗，病人康复了,但类似的发作在4个月后复发，没有任何特定的触发因素。根据两次发作的临床表现和实验室检查结果，他被诊断为ISCLS。通过补充氧气和使用生理盐水进行大量置换可缓解症状，并每天一次给患者开10mg班布特罗和400mg茶碱。他在住院第5天出院。此后,即使在COVID-19感染的情况下，患者也已随访5年，没有任何ISCLS症状或复发。
结论：ISCLS是一种极罕见且经常被误诊的疾病。然而,早期诊断,通过积累的临床数据进行治疗和预防可以预防ISCLS复发和相关致命并发症的发展。因此,临床医生需要充分了解本病的各种临床特征和治疗方案.
