From the Research
Diuretics are not the primary treatment for resolving systemic capillary leak syndrome, and their use may even be contraindicated due to the risk of further reducing intravascular volume and worsening hypotension. Systemic capillary leak syndrome (SCLS) is a rare condition characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration due to the leakage of plasma from blood vessels into surrounding tissues 1. The standard treatment approach for acute episodes includes aggressive fluid resuscitation with intravenous fluids (typically albumin and crystalloids) to maintain adequate circulation, along with vasopressors if needed for blood pressure support. For long-term prevention of episodes, monthly intravenous immunoglobulin (IVIG) at 1-2 g/kg is the most effective prophylactic therapy 2. Some patients may benefit from theophylline (400-800 mg/day) and terbutaline (5-10 mg/day) as preventive measures.
The use of diuretics in SCLS is generally not recommended, as they can exacerbate the condition by further reducing intravascular volume and worsening hypotension 3. However, in certain cases, such as in neonates and infants with diuretic-refractory CLS after cardiac surgery, additional aquaretic therapy with Tolvaptan may be considered 4. It is essential to note that the primary goal in managing SCLS is to maintain adequate circulation and prevent organ dysfunction, rather than solely focusing on reducing edema.
Key points to consider in the management of SCLS include:
- Aggressive fluid resuscitation with intravenous fluids to maintain adequate circulation
- Use of vasopressors if needed for blood pressure support
- Monthly intravenous immunoglobulin (IVIG) at 1-2 g/kg for long-term prevention of episodes
- Potential use of theophylline and terbutaline as preventive measures
- Avoidance of diuretics, except in specific cases where aquaretic therapy may be beneficial.
Overall, the management of SCLS requires a comprehensive approach that prioritizes maintaining adequate circulation and preventing organ dysfunction, rather than solely focusing on reducing edema with diuretics 1.