Severe capillary leak syndrome with cardiac arrest triggered
Systemic capillary leak syndrome (SCLS), also known as Clarkson syndrome, is a rare disease with potential fatal outcome. The clinical picture involves leakage of fluid and protein from the bloodstream into peripheral tissues, resulting in hypoalbuminaemia, elevated haematocrit, oedema and hypotension.

The spectrum of the symptoms ranges from discrete swelling/oedema of extremities to fulminant cardiogenic shock.

Published in BMJ, the authors present a case with a 52-year-old man diagnosed with SCLS after being resuscitated from cardiac arrest, which was complicated by compartment syndrome. The severe episode of capillary leak was potentially triggered by influenza virus infection. With the benefit of hindsight, he presented with symptoms of SCLS 2 years prior the major acute episode.

The authors describe this case and review some aspects of the literature on SCLS, with particular focus on the pathogenesis, treatment/prophylaxis and long-term physical and psychological complications.

Learning points
• Localised oedema of unknown origin or episodes of increased haematocrit of unknown origin, consider systemic capillary leak syndrome (SCLS).

• In confirmed SCLS, IVIG prophylaxis (1–2 g/kg/month) is the standard of care.

•SCLS may associated with several clinical symptoms and sequelae, including fatigue, muscle pain, depression and recurrent episodes with minor or major leaks.

• Better understanding of the genetics and immunology underlying the exaggerated microvascular endothelial response to inflammation and physical stress leading to capillary leak syndrome may improve treatment and prophylaxis of the disease.

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