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Rare disease
High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome
  1. Manuel Zipponi,
  2. Roland Eugster,
  3. Tanja Birrenbach
  1. Department of General Internal Medicine, Inselspital Bern, University Hospital Bern, Bern, Switzerland
  1. Correspondence to Dr Tanja Birrenbach, tbirrenbach{at}


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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  • Competing interests None.

  • Patient consent Obtained.

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