Syndrome de fuite capillaire idiopathique et formes secondaires : une revue systématique de la littérature

Gemcitabine-induced chronic systemic capillary leak syndrome

“…Dr Clarkson first described it in 1960 and since then both case reports and case series have been published in literature. 1 7 The exact incidence of the disorder is unknown as most cases go undiagnosed. The incidence seems to have increased in the past 15 years, due to increased awareness and successful identification of the disease with the help of classic laboratory findings.…”

“…It can be idiopathic (Clarkson’s disease) or secondary to various conditions, such as monoclonal gammopathy of undetermined significance, engraftment syndrome after haematopoietic stem cell transplantation, viral infections (eg, viral haemorrhagic fever like hantavirus, dengue haemorrhagic fever), snake bites and secondary to treatments, such as chemotherapy medications. 1 2 The pathophysiology is thought to be secondary to derangements in the endothelial surface causing leakage of proteins and plasma into the interstitial compartment. High levels of plasma vascular endothelial growth factor (VEGF) levels have been reported during acute, severe episodes.…”

Severe cerebral involvement due to idiopathic systemic capillary leak syndrome

“…Idiopathic systemic capillary leak syndrome (ISCLS) is a rare and life‐threatening disorder first described by Clarkson and is characterized by recurrent attacks of hypotension, hypoalbuminemia, and hemoconcentration 1, 2, 3. The cause of ISCLS is unknown.…”

What else ? Dyspnée soudaine chez une femme de 45 ans

“…L'absence de maladie aiguë du système nerveux central permettait d'écarter un oedème pulmonaire neurogène. Un syndrome de Clarkson (syndrome de fuite capillaire systémique) récidivant aurait entraîné des oedèmes diffus, une hémoconcentra-tion et une hypoalbuminémie [4]. Une mastocytose systémique se compliquant d'une hypotension paroxystique sévère n'aurait pas expliqué l'oedème pulmonaire.…”