The systemic capillary leak syndrome: a scarcely known nephrological entity.

[systemic capillary leak syndrome]

The idiopathic systemic capillary leak syndrome (SCLS ) is a rare life-threatening disorder characterized by periodic episodes of hypovolemic shock , due to plasma leakage from the intravascular to the interstitial space , as reflected by accompanying hypoalbuminemia , hemoconcentration and edema . Here we report the case of a 65 -year -old woman affected by SCLS who required aggressive resuscitation with norepinephrine , steroids , albumin and crystalloids . Then , a long -term prophylaxis with a ß (2 )-adrenergic receptor agonist and theophylline was started . In conclusion , though SCLS is a rare entity , the associated morbidity and mortality require the physician 's awareness to provide timely therapy . Underrecognition in the medical community and rarity of this syndrome have precluded analysis by rational clinical trial designs that are necessary to determine more targeted and adequate therapy . This report is meant to enhance awareness of SCLS in the nephrology community .