High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome.
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.
Department of General Internal Medicine, Inselspital Bern, University Hospital Bern, Bern, Switzerland.
Manuel
M
Zipponi
Roland
R
Eugster
Tanja
T
Birrenbach
D000328:Adult; D019559:Capillary Leak Syndrome / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome
D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous