Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review

Noor Ul Ain Baloch, Marvi Bikak, Abdul Rehman, Omar Rahman

Research output: Contribution to journalReview articlepeer-review

17 Scopus citations


Introduction: Idiopathic systemic capillary leak syndrome (SCLS) is a unique disorder characterized by episodes of massive systemic leak of intravascular fluid leading to volume depletion and shock. A typical attack of SCLS consists of prodromal, leak and post-leak phases. Complications, such as compartment syndrome and pulmonary edema, usually develop during the leak and post-leak phases respectively. Judicious intravenous hydration and early use of vasopressors is the cornerstone of management in such cases. Areas covered: The purpose of the present review is to provide an up-to-date, evidence-based review of our understanding of SCLS and its management in the light of currently available evidence. Commentary: Idiopathic SCLS was first described in 1960 and, since then, more than 250 cases have been reported. A large number of cases have been reported over the past one decade, most likely due to improved recognition. In the acute care setting, most patients with SCLS are managed as per the Surviving Sepsis guidelines and receive aggressive volume resuscitation–which is not the optimal management strategy for such patients. There is a need to raise awareness amongst physicians and clinicians in order to improve recognition of this disorder and ensure its appropriate management.

Original languageEnglish
Pages (from-to)331-340
Number of pages10
JournalExpert Review of Cardiovascular Therapy
Issue number5
StatePublished - 4 May 2018
Externally publishedYes


  • Systemic capillary leak syndrome
  • compartment syndrome
  • edema
  • hypotension
  • shock


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