Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment

Am J Med. 1997 Dec;103(6):514-9. doi: 10.1016/s0002-9343(97)00272-6.


Background: Systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse swelling, weight gain, and renal shut-down. From the first publication in 1960, only 34 cases have been reported.

Objective: To collate enough patients to observe the natural history of the disease and evaluate the efficacy of empiric treatments.

Design: Multicentric retrospective study.

Results: Thirteen patients (6 women and 7 men) were collated with a mean follow-up of 6.4 years. Eight patients are still alive after a mean of 5.6 years (range 1 to 15). Three patients out of the 11 who were not lost to follow-up died; 1 during an attack and 2 because of a progression towards multiple myeloma.

Conclusions: Our series shows an improvement in the prognosis of SCLS due most likely to improved management during attacks. Some patients' disease could evolve into a multiple myeloma. Treatment is still empiric and no prophylactic therapy, including terbutaline associated with aminophylline, has clearly proven its efficacy.

Publication types

  • Case Reports
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Adult
  • Capillary Leak Syndrome* / complications
  • Capillary Leak Syndrome* / physiopathology
  • Capillary Leak Syndrome* / therapy
  • Chronic Disease
  • Disease Progression
  • Female
  • Humans
  • Male
  • Middle Aged
  • Multiple Myeloma / etiology
  • Retrospective Studies