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Narrative review: the systemic capillary leak syndrome
Kirk M Druey1, Philip R Greipp
1National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, and Mayo Clinic College of Medicine, Rochester, Minnesota 20892, USA. kdruey@niaid.nih.gov
Annals of Internal Medicine
|July 21, 2010
Summary
Systemic capillary leak syndrome (SCLS) is a rare, life-threatening condition causing plasma leakage and vascular collapse. Early diagnosis and management are crucial for survival, with potential for long-term recovery.
Area of Science:
- Vascular Biology
- Rare Diseases
- Internal Medicine
Background:
- Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent episodes of plasma extravasation and vascular collapse.
- The exact etiology of SCLS remains unknown, but it is hypothesized to involve transient endothelial dysfunction.
- SCLS is likely underdiagnosed due to nonspecific symptoms, leading to delayed treatment and high mortality rates.
Purpose of the Study:
- To provide a comprehensive overview of systemic capillary leak syndrome.
- To highlight diagnostic challenges and clinical manifestations.
- To discuss current treatment strategies and prognosis.
Main Methods:
- Clinical case review and literature synthesis.
- Diagnostic criteria based on characteristic symptoms and exclusion of other conditions.
- Review of therapeutic interventions and patient outcomes.
Main Results:
- SCLS presents with shock, massive edema, hemoconcentration, and hypoalbuminemia.
- Patients are at risk for organ failure, rhabdomyolysis, and thromboembolism during acute episodes.
- Rapid fluid remobilization can lead to fatal flash pulmonary edema.
Conclusions:
- Diagnosis of SCLS requires clinical suspicion in patients with unexplained edema, hypotension, and hemoconcentration.
- Prompt management with vasopressors and fluid replacement is critical.
- While prognosis is uncertain, long-term survival rates can exceed 70% for those who recover from severe episodes.
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