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


METHODS
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.
Amoura
Z
Z
Service de Médecine Interne, Hôpital de la Pitié, Paris, France.
Papo
T
T
Ninet
J
J
Hatron
P Y
PY
Guillaumie
J
J
Piette
A M
AM
Blétry
O
O
Dequiedt
P
P
Talasczka
A
A
Rondeau
E
E
Dutel
J L
JL
Wechsler
B
B
Piette
J C
JC
D000208:Acute Disease; D000328:Adult; D019559:Capillary Leak Syndrome / Q000150:complications / Q000503:physiopathology / Q000628:therapy; D002908:Chronic Disease; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000209:etiology; D012189:Retrospective Studies