Article Text

PDF
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}yahoo.de

Summary

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.

Statistics from Altmetric.com

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.