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Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. A case series.
1Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Systemic capillary leak syndrome (SCLS) prophylaxis with terbutaline and theophylline significantly reduced episode frequency and severity. Maintaining therapeutic drug levels is crucial for favorable outcomes in SCLS patients.
Area of Science:
- Internal Medicine
- Pharmacology
Background:
- Systemic capillary leak syndrome (SCLS) is a rare, idiopathic disorder with high mortality.
- Characterized by recurrent hypotension and hemoconcentration due to plasma extravasation.
Observation:
- A case series evaluated eight SCLS patients over 18 years.
- Intervention involved oral terbutaline combined with aminophylline or theophylline.
Findings:
- Prophylaxis with terbutaline and theophylline reduced SCLS episode frequency and severity by 30-fold.
- Two of eight patients died; six remained healthy with reduced episodes.
- Extended-release formulations and maintained therapeutic theophylline levels correlated with better outcomes.
Implications:
- Terbutaline and theophylline offer effective prophylaxis for systemic capillary leak syndrome.
- Monitoring and maintaining therapeutic drug levels are essential for managing SCLS.
- Further research into optimal drug formulations and management strategies is warranted.
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