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Idiopathic Systemic Capillary Leak Syndrome
Kashmira Rajendra Hajare1, Priya Patil1, Jyoti Bansode1
1Department of Medicine, GGMC and JJ Group of Hospitals, Mumbai, Maharashtra, India.
Summary
Idiopathic systemic capillary leak syndrome (ISCLS) is a rare, life-threatening condition. Long-term prophylaxis with beta-2 adrenergic agonists and theophylline managed recurrent capillary leak episodes in a case report.
Area of Science:
- Internal Medicine
- Hematology
- Critical Care Medicine
Background:
- Idiopathic systemic capillary leak syndrome (ISCLS) is a rare, life-threatening disorder.
- Characterized by recurrent episodes of hypotension, hemoconcentration, and hypoalbuminemia due to endothelial dysfunction and plasma leakage.
Observation:
- A case of ISCLS with recurrent capillary leak episodes is presented.
- The patient required long-term prophylaxis with a beta-2 adrenergic receptor agonist and theophylline.
Findings:
- This case highlights the potential benefit of beta-2 adrenergic agonists and theophylline in managing recurrent ISCLS.
- Demonstrates a therapeutic approach for a rare condition with significant morbidity and mortality.
Implications:
- Increased physician awareness of ISCLS is crucial for timely diagnosis and treatment.
- Further research is needed to establish targeted and adequate therapies for ISCLS due to its rarity.
Keywords:
Agonistbeta-2 receptorendothelial dysfunctionhemoconcentrationhypoalbuminemiahypotensionidiopathic systemic capillary leak syndrometheophyllineMore Related Videos
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