title: [Idiopathic capillary leak syndrome].
abstract: Idiopathic capillary leak syndrome (ICLS) is a rare and poorly known condition. Since the first description in 1960, about hundred cases have been reported. A French register that was initiated in 1997 provides a better knowledge of the natural course of the disease and highlights some therapeutic issues. ICLS mainly affects middle-aged adults. The prognosis is poor with a 10-year mortality rate around 34%. Severe crisis and complications occurring in intensive care units account for 80% of the mortality. Diagnosis relies on an almost pathognomonic association: recurrent attacks of hypotension and hemoconcentration with paradoxical hypoalbuminemia. A monoclonal gammopathy is found in about 80% of patients. Physiopathology still remains unclear. Paraprotein toxicity has never been demonstrated. As a result, no evidence-based treatment is available neither for acute crisis nor for prophylaxis. Management of acute episodes is mainly symptomatic. Fluid infusion must be cautious because it can induce complications during the recovery phase. Patient education is a major measure to prevent recurrent attacks. Beta-2 stimulants were reported to reduce the frequency and severity of episodes and were considered as the recommended prophylactic measure. However, early data from the French register suggest that intravenous immunoglobulins are more effective in reducing both frequency and severity of the attacks. Inclusion of patients with ICLS in a register is crucial to improve the knowledge about aetiology and treatment of this disorder.
authors.affiliation: Service de médecine interne, Centre national de références maladies rares, hôpital de la Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Gousseff
authors.forename: Z
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000517:prevention & control; D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010353:Patient Education as Topic; D011379:Prognosis; D012042:Registries; D013997:Time Factors
chemical_list: D004232:Diuretics; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors