[Idiopathic systemic capillary leak syndrome: 2 cases with misleading presentation].

Idiopathic systemic capillary leak syndrome (ISCLS) also known as Clarkson syndrome is a rare and sudden life-threatening entity. Three consecutive phases are described. A first non-specific prodromal phase often manifests as "flu-like" symptoms and precedes capillary leak phase with major hypovolemic and distributive shock leading to serious and frequent multiorgan dysfunction syndrome (MODS). Severe hypovolemia contrasts with edema, and hemoconcentration with hypoalbuminemia. ISCLS is characterized by these two clinical and biological paradoxes. Subsequent recovery phase exhibits organ function restoration along with interstitial/intravascular volumes normalization. The latter occurs spontaneously and systematically in patients surviving from leak phase. We report here two ISCLS cases admitted in intensive care unit (ICU) both enhancing initial misdiagnosis possibly lowering prognosis and outcome. Our first 28-year-old female patient was admitted for « polycythemia vera » although hemoconcentration was attributable to hypovolemia. She presented circulatory arrest during the second bloodletting session and complicated with MODS. In and out ICU favorable outcome was noted on intravenous immunoglobulin therapy. A second 57-year-old male patient was admitted in ICU for severe "myositis" (myalgia and rhabdomyolysis) although rectified diagnosis retained compartment syndrome (muscular severe edema following capillary leak). Rapid and refractory hypovolemic shock appeared with subsequent MODS leading to death. ISCLS pathophysiology remains unknown but certainly implies transitory endothelial dysfunction. Impossibility of randomized controlled trial for this exceptional disease led to based-on-experience therapeutic guidelines implying symptomatic care (cardiac output surveillance, nephroprotection, prudent fluid intake, prudent vasoactive amine use) and specific therapies (intravenous aminophylline during severe flares). Although enhancing controversial and even deleterious effects during the acute phase, polyvalent immunoglobulins are effective for relapse prevention. Syndromic diagnosis is difficult, but its precocious finding constitutes a key-element in better outcome before organ failure.

Bichon A ，Carvelli J ，Bourenne J ，Gainnier M ，Harlé JR ，Schleinitz N ... -  《-》

A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report.

Hirosaki Y ，Hayashidani S ，Ouchi S ，Ohshima T ，Nakano R ，Yamamoto H ... -  《-》

Idiopathic systemic capillary leak syndrome presenting as septic shock: A case report.

Idiopathic capillary leak syndrome (Clarkson's Disease) is a rare angiopathy with a heterogenous phenotype that may present as distributive shock refractory to resuscitative management. We report a case of idiopathic systemic capillary leak syndrome presenting as septic shock. Structured case report and review of the literature. A 27-year old man admitted to our institution with coryzal symptoms rapidly deteriorated with presumed sepsis, leading to intensive care unit admission. Following further deterioration, Idiopathic systemic capillary leak syndrome was considered and intravenous immunoglobulin administered, resulting in rapid improvement in the patient's clinical status. Idiopathic systemic capillary leak syndrome is a rare and potentially life-threatening angiopathy that may present as, and should be considered in, refractory distributive shock. Administration of intravenous immunglobulin resulted in rapid recovery in this patient, and has been associated with positive outcomes in previous cases.

Raith EP ，Ihle JF ，Jamieson J ，Kalff A ，Bosco J ... -  《-》

10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report.

Iwasa T ，Ohashi H ，Kihira K ，Koike Y ，Otake K ，Inoue M ，Sawada H ，Toyoda H ，Komada Y ... -  《BMC Pediatrics》

A case report of systemic capillary leak syndrome (Clarkson's disease).

Hollenberg J ，Frykman J ，Lundberg LG ，Forsberg S ... -  《-》