From the Research
Diagnosis of Systemic Capillary Leak Syndrome (SCLS)
- SCLS is characterized by increased capillary permeability to proteins and fluid extravasation from blood vessels into surrounding tissues and body cavities, leading to hypotension, generalized anasarca, pleural effusions, and pericardial effusions 1
- Diagnosis of SCLS can be challenging and is sometimes a diagnosis of exclusion, requiring the exclusion of secondary diseases, especially when a serum monoclonal immunoglobulin is present, or when there is a relapsing disease, no initial lung involvement, or preserved consciousness despite low blood pressure 2
- Clinical presentation of SCLS includes hypotension, edema, hemoconcentration, and hypoalbuminemia, and can be misdiagnosed as hypovolemic shock, septic shock, polycythemia vera, or angioedema 3
Treatment of Systemic Capillary Leak Syndrome (SCLS)
- Treatment of SCLS includes corticosteroids, diuretics, albumin, immunoglobulins, and crystalloids 1
- Acute episodes of SCLS are treated with vasopressor therapy and judicious fluid replacement, while patients with Clarkson's disease may be treated with intravenous immunoglobulins between episodes 2
- Intensive care and supportive therapy constitute the mainstay of the treatment, along with judicious use of crystalloids and colloids such as dextran and starch during the leak phase 4
- The use of proning, steroids, and intravenous immunoglobins may be beneficial in patients with SCLS, and extracorporeal membrane oxygenation appears to be an excellent strategy to surmount the impediments of the leak and post-leak phase of SCLS, especially in patients with severe or refractory hypoxemia 4
- Prophylactic treatment with β2 agonists, methylxanthines, and intravenous immunoglobulins may improve survival rates in patients with SCLS 3