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[Idiopathic systemic capillary leak syndrome: 2 cases with misleading presentation]
A Bichon1, J Carvelli2, J Bourenne2
1Service de médecine interne, hôpital de la Timone, AP-HM, 264, rue Saint-Pierre, 13005 Marseille, France; Université d'Aix-Marseille, Marseille, France.
La Revue De Medecine Interne
|April 13, 2021
Summary
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare, life-threatening condition. Early diagnosis and treatment, including intravenous immunoglobulin therapy, are crucial for improving patient outcomes and preventing multi-organ dysfunction syndrome.
Area of Science:
- Critical Care Medicine
- Rare Diseases
- Internal Medicine
Background:
- Idiopathic systemic capillary leak syndrome (ISCLS), or Clarkson syndrome, is a rare, acute, and life-threatening condition.
- ISCLS presents with three phases: prodromal, capillary leak (causing hypovolemic shock and multi-organ dysfunction syndrome), and recovery.
- Key paradoxes include severe hypovolemia with edema and hemoconcentration with hypoalbuminemia.
Observation:
- Two ISCLS cases highlight the critical impact of misdiagnosis on prognosis.
- A misdiagnosed case of "polycythemia vera" led to circulatory arrest and multi-organ dysfunction syndrome, but responded to intravenous immunoglobulin therapy.
- Another misdiagnosed case of "myositis" resulted in fatal hypovolemic shock and multi-organ dysfunction syndrome.
Findings:
- Early and accurate diagnosis of ISCLS is essential for effective management.
- Intravenous immunoglobulin therapy shows promise for relapse prevention in ISCLS.
- Symptomatic care, including hemodynamic monitoring and organ support, is vital during acute flares.
Implications:
- Despite unknown pathophysiology, prompt diagnosis and management of ISCLS can significantly improve outcomes.
- Further research into ISCLS pathophysiology may reveal novel therapeutic targets.
- Raising awareness of ISCLS among clinicians is crucial to avoid diagnostic delays and improve patient survival.
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