There is currently no cure for systemic capillary leak syndrome (SCLS). Treatment for SCLS during an episode is mainly supportive, aiming to stabilize symptoms and prevent severe complications. This may involve stabilizing the airway and breathing, taking certain medications, and/or intravenous (IV) infusion of fluids, medications, or blood products. Some have suggested that IV fluids are not recommended because it may worsen swelling (edema) or increase the risk for other complications. Once a person begins to recover, diuretics are almost always required.
Periodic infusions or the use of certain medications are used to try to prevent future episodes (prophylactic therapy). Having monthly infusions of intravenous immune globulin (IVIG) has been suggested. A study published in 2017 found that preventive treatment with IVIG was the strongest factor associated with survival in people with SCLS. Those who do not improve with IVIG or who cannot tolerate the therapy may have success with theophylline and terbutaline.
Research into how SCLS develops and factors that affect its course are underway, in hopes that a better understanding of SCLS will lead to more effective treatment options and a lower mortality rate.
Last updated: 7/31/2017
We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please
Pineton de Chambrun M, Gousseff M, Mauhin W, et al. Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. Am J Med. June 9, 2017; https://www.ncbi.nlm.nih.gov/pubmed/28602874.