Author/Authors :
Zahir Amoura MD، نويسنده , , Thomas Papo MD، نويسنده , , Jacques Ninet MD، نويسنده , , Pierre-Yves Hatron MD، نويسنده , , Jacques Guillaumie MD، نويسنده , , Anne-Marie Piette MD، نويسنده , , Olivier Blétry MD، نويسنده , , Philippe Dequiedt MD، نويسنده , , Aline Talasczka MD، نويسنده , , Eric Rondeau MD، نويسنده , , Jean Louis Dutel MD، نويسنده , , Bertrand Wechsler MD، نويسنده , , Jean-Charles Piette MD، نويسنده ,
Abstract :
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.
