title: The systemic capillary leak syndrome: a case series of 28 patients from a European registry.
abstract: BACKGROUND
  The systemic capillary leak syndrome (SCLS) is a rare disease characterized by life-threatening attacks of capillary hyperpermeability.
  
  
  OBJECTIVE
  To describe the clinical characteristics, laboratory findings, treatments, and outcomes of patients with SCLS who were not previously reported in the literature.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Patients referred to a European multicenter SCLS registry between January 1997 and July 2010.
  
  
  METHODS
  28 patients with SCLS.
  
  
  METHODS
  Frequency, severity of attacks, and vital status were assessed every 6 months, from diagnosis to the end of the study.
  
  
  RESULTS
  13 men and 15 women referred to the registry who were not previously reported in the literature had 252 attacks. Median age at disease onset was 49.1 years (range, 5.4 to 77.7 years), and median annual frequency of attacks was 1.23 (range, 0.13 to 21.18) per patient. Monoclonal IgG gammopathy was observed in 25 patients (89%). Preventive treatment included intravenous immunoglobulin (n = 18), terbutaline (n = 9), and aminophylline (n = 10). Eight patients died (29%); 1-year survival was 89%, and 5-year survival was 73%. Death was directly related to SCLS attacks in 6 of 8 cases (75%). In 10 patients with a prediagnosis period greater than 6 months who received preventive treatment, the annual frequency of attacks after diagnosis decreased by a median of 1.55 (range, 0.14 to 8.84) per patient. Five years after diagnosis, survival was 85% in 23 patients who had received prophylactic treatment and 20% in 5 patients who had not.
  
  
  CONCLUSIONS
  The benefits of preventive treatment could not be precisely ascertained because of the small sample size and because most patients received several treatments.
  
  
  CONCLUSIONS
  Clinical experience with these 28 patients with SCLS suggests that prophylactic treatment with β(2)-agonists or intravenous immunoglobulin may reduce the frequency and severity of attacks and may improve survival.
  
  
  BACKGROUND
  Université Pierre et Marie Curie, Paris, France.
authors.affiliation: Service de médecine interne 2, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l'Hôpital, 75013 Paris, France.
authors.forename: Marie
authors.initials: M
authors.lastname: Gousseff
authors.forename: Laurent
authors.initials: L
authors.lastname: Arnaud
authors.forename: Marc
authors.initials: M
authors.lastname: Lambert
authors.forename: Arnaud
authors.initials: A
authors.lastname: Hot
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.forename: Pierre
authors.initials: P
authors.lastname: Duhaut
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.forename: Martin
authors.initials: M
authors.lastname: Soubrier
authors.forename: Marc
authors.initials: M
authors.lastname: Ruivard
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Malizia
authors.forename: Nathalie
authors.initials: N
authors.lastname: Tieulié
authors.forename: Sophie
authors.initials: S
authors.lastname: Rivière
authors.forename: Jacques
authors.initials: J
authors.lastname: Ninet
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000293:Adolescent; D000318:Adrenergic beta-Agonists / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D012042:Registries; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000318:Adrenergic beta-Agonists; D002121:Calcium Channel Blockers; D016756:Immunoglobulins, Intravenous