[Idiopathic systemic capillary leak syndrome: a case report with cardiac involvement].

Systemic capillary leak syndrome (SCLS) is characterized by recurrent hypovolemic shock associated with edema, hemoconcentration and paradoxal hypoprotidemia due to leakage into the interstitial space. We report a new case with cardiac involvement. A 62-year-old man had two recurrent hypovolemic shocks with SCLS's criteria, associated twice with cardiac systolic dysfunction and atrial fibrillation. This report should enhance physician awareness of SCLS, which is a rare and severe disease, and its cardiac involvement.

Guillaume M ，Tolsma V ，Colombe B ，Bosseray A ，Massot C ... -  《-》

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

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

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: 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 ... -  《-》

Systemic capillary leak syndrome.

Chihara R ，Nakamoto H ，Arima H ，Moriwaki K ，Kanno Y ，Sugahara S ，Okada H ，Suzuki H ... -  《-》