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Genetic and Rare Diseases Information Center (GARD)

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Systemic capillary leak syndrome


Other Names for this Disease
  • Capillary leak syndrome
  • Capillary leak syndrome with monoclonal gammopathy
  • Clarkson disease
  • Periodic systemic capillary leak syndrome
  • SCLS
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Overview



What is systemic capillary leak syndrome?

How might systemic capillary leak syndrome be treated?


What is systemic capillary leak syndrome?

Systemic capillary leak syndrome is a condition in which fluid and proteins leak out of tiny blood vessels and flow into surrounding tissues, resulting in dangerously low blood pressure.[1] Attacks frequently last for several days and require emergency care.[2] Most cases of capillary leak occur randomly in previously healthy adults.[3] Treatment involves preventing attacks using medications which may decrease capillary leakage and interfere with hormones that may cause future leakage. Once an attack is underway, treatment is aimed at controlling blood pressure to maintain blood flow to vital organs and prevention of swelling due to fluid accumulation.[2] Capillary leak syndrome may lead to multiple organ failure, shock and even death. [1][2]
Last updated: 8/30/2012

How might systemic capillary leak syndrome be treated?

Unfortunately, there is no cure for systemic capillary leak syndrome at this time.  However, recent studies suggest that taking medication known as beta-adrenergic agonists (including terbutaline) or undergoing immunoglobulin intravenous (IV) therapy may reduce the frequency of attacks and may increase survival in individuals affected with this condition.[4]
Last updated: 8/30/2012

References
  1. Dictionary of Cancer Terms: capillary leak syndrome. National Cancer Institute (NCI). http://www.cancer.gov/dictionary?CdrID=426421. Accessed 8/27/2012.
  2. Greipp PR. Systemic Capillary Leak Syndrome. National Organization for Rare Disorders (NORD). 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/1106/viewAbstract. Accessed 8/27/2012.
  3. Druey KM, Greipp PR. Narrative Review: The Systemic Capillary Leak Syndrome. Annals of Internal Medicine. 2010; http://www.annals.org/content/153/2/90.full.pdf+html. Accessed 8/27/2012.
  4. Gousseff M, Arnaud L, Lambert M, Hot A, Hamidou M, Duhaut P, Papo T, Soubrier M, Ruivard M, Malizia G, Tieulié N, Rivière S, Ninet J, Hatron PY, Amoura Z; Capillary Leak Syndrome Registry. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Annals of Internal Medicine. 2011; 154:464-471. http://www.ncbi.nlm.nih.gov/pubmed/21464348. Accessed 8/28/2012.