Department of Biomedical and Clinical Sciences "Luigi Sacco", ASST Fatebenefratelli Sacco, Luigi Sacco Hospital, University of Milan, Via Giovanni Battista Grassi, 74, 20157, Milan, Italy.
Intensive Care Unit, ASST Fatebenefratelli Sacco, Milan, Italy.
Intern Emerg Med. 2019 Aug;14(5):723-730. doi: 10.1007/s11739-019-02113-4. Epub 2019 Jun 1.
Idiopathic systemic capillary leak syndrome (ISCLS) presents with recurrent potentially life-threatening episodes of hypovolemic shock associated with severe hemoconcentration and hypoproteinemia. Timely recognition is of paramount importance because ISCLS, despite resembling other kinds of hypovolemic shock, requires a peculiar approach, to prevent life-threatening iatrogenic damage. Due to the rarity of this condition with only scattered cases described worldwide, evidence-based recommendations are still lacking. Here, we summarize our 40 years' experience in treating shock in ISCLS patients to derive a therapeutic algorithm. Records from 12 ISCLS patients (mean follow-up is 6 years, with a mean age at symptoms' onset of 51.5 years) were informative for treatment modalities and outcome of 66 episodes of shock. Episodes are divided in three phases and treatment recommendations are the following: prodromal symptoms-signs (growing malaise, oligo-anuria, orthostatic dizziness) last 6-12 h and patients should maintain rigorous bed rest. The acute shock phase lasts 24-36 h. Patients should be admitted to ICU, placed on restrictive infusion of fluids favoring cautious boluses of high-molecular-weight plasma expanders when SAP < 70 mmHg; monitored for cerebral/cardiac perfusion, myocardial edema and signs of compartment syndrome. The post-acute (recovery) phase may last from 48 h to 1 week. Monitor for cardiac overload to prevent cardiac failure; in case of persistent renal failure, hemodialysis may be necessary; consider albumin infusion. Complications listed by frequency in our patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema, pericardial-pleural-abdominal effusion, cerebral involvement, acute pulmonary edema and deep vein thrombosis.
特发性全身性毛细血管渗漏综合征（ISCLS）表现为反复发作的、可能危及生命的低血容量性休克，伴有严重的血液浓缩和低蛋白血症。及时识别非常重要，因为 ISCLS 虽然类似于其他类型的低血容量性休克，但需要一种特殊的方法来预防危及生命的医源性损伤。由于这种情况非常罕见，全球只有零星病例描述，因此仍然缺乏循证推荐。在这里，我们总结了我们在治疗 ISCLS 患者休克方面 40 年的经验，得出了一个治疗算法。12 名 ISCLS 患者的记录（平均随访时间为 6 年，症状发作时的平均年龄为 51.5 岁）对休克的治疗方式和 66 例休克的结果有提示意义。发作分为三个阶段，治疗建议如下：前驱症状-体征（逐渐恶化的不适、少尿、直立性头晕）持续 6-12 小时，患者应保持严格卧床休息。急性休克期持续 24-36 小时。患者应入住 ICU，限制液体输注，当 SAP<70mmHg 时，优先谨慎给予高相对分子质量的血浆扩容剂；监测脑/心灌注、心肌水肿和间隙综合征的迹象。急性后（恢复）期可持续 48 小时至 1 周。监测心脏负荷过重以预防心力衰竭；如果持续肾衰竭，可能需要血液透析；考虑白蛋白输注。我们的患者中列出的并发症按频率依次为急性肾衰竭、间隙综合征和神经病、横纹肌溶解、心肌水肿、心包-胸腔-腹腔积液、脑受累、急性肺水肿和深静脉血栓形成。
