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BMJ Case Rep. 2011 May 3;2011. pii: bcr1220103599. doi: 10.1136/bcr.12.2010.3599.

High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome.

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  • 1Department of General Internal Medicine, Inselspital Bern, University Hospital Bern, Bern, Switzerland.


The systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a life-threatening disorder of unknown cause. It is characterised by recurrent acute episodes of hypotension, weight gain and generalised oedema with haemoconcentration and hypoproteinaemia caused by paroxysmal capillary hyperpermeability with a shift of plasma fluid from the intravascular to the interstitial space. We report the case of a 40-year-old woman with chronic SCLS treated with high-dose intravenous immunoglobulins, after a prophylactic therapy with theophylline and terbutaline was poorly tolerated and failed to decrease the frequency and severity of the attacks sufficiently.

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