title: [Idiopathic and secondary capillary leak syndromes: A systematic review of the literature].
abstract: OBJECTIVE
  The capillary leak syndrome (CLS) is a rare condition characterized by the onset of hypotension, edema, hemoconcentration and hypoalbuminemia. CLS can be idiopathic (Clarkson's disease) or secondary to various conditions and treatments. Here, we review the clinical and biological features, pathophysiology, causes and treatment of this rare condition.
  
  
  METHODS
  We performed a systematic review of the literature (Medline database through February 2014) to identify all articles about CLS. The relevant references were selected by two independent authors.
  
  
  RESULTS
  Secondary CLSs are mostly due to malignant hematological diseases, viral infections, and treatments such as chemotherapies and therapeutic growth factors. Diagnosis of idiopathic CLS is made by exclusion of secondary diseases, especially as a serum monoclonal immunoglobulin is present, or when there is a relapsing disease, no initial lung involvement or preserved consciousness despite low blood pressure. Acute episodes are treated with vasopressor therapy and judicious fluid replacement. Between episodes, patients with Clarkson's disease may be treated with intravenous immunoglobulins. CLS is a severe disease with significantly impaired prognosis.
  
  
  CONCLUSIONS
  Clarification of the pathophysiological mechanisms of CLS is essential to improve the prognosis of this rare disease with more targeted treatments.
authors.affiliation: Service de médecine interne 2, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Duron
authors.affiliation: Service de médecine interne 2, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Delestre
authors.affiliation: Service de médecine interne 2, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France; UPMC université Paris 06, Sorbonne universités, 75013 Paris, France.
authors.forename: Z
authors.initials: Z
authors.lastname: Amoura
authors.affiliation: Service de médecine interne 2, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France; UPMC université Paris 06, Sorbonne universités, 75013 Paris, France. Electronic address: laurent.arnaud@psl.aphp.fr.
authors.forename: L
authors.initials: L
authors.lastname: Arnaud
mesh_terms: D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D011379:Prognosis
keywords: Capillary leak syndrome; Clarkson; Clarkson's disease; Revue systématique; Syndrome de fuite capillaire; Systematic review