High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome.
OBJECTIVE
The objective of this study was to report the dramatic improvement of patients with systemic capillary leak syndrome obtained with high-dose intravenous immunoglobulins.


METHODS
Systemic capillary leak syndrome is a rare and life-threatening disorder characterized by hypotension that can lead to shock, weight gain, hypoalbuminemia, and elevated hematocrit secondary to unexplained episodic capillary fluid extravasation into the interstitial space. Because its cause is unknown, systemic capillary leak syndrome treatment has remained largely supportive.


RESULTS
Intravenous immunoglobulins administration to a patient with refractory systemic capillary leak syndrome yielded dramatic improvement. The patient is still alive 11 yrs after systemic capillary leak syndrome diagnosis and receives intravenous immunoglobulins monthly. Later, based on that result, intravenous immunoglobulins were successfully given to two other patients during the acute phase of systemic capillary leak syndrome. Both are still alive 8 and 1.5 yrs after receiving intravenous immunoglobulins at the onset of each flare.


CONCLUSIONS
Intravenous immunoglobulins were effective against systemic capillary leak syndrome symptoms in three patients, but their exact mechanism remains unknown. Their immunomodulatory effect merits further investigation.
Department of Internal Medicine and Faculty of Medicine, University of Lille II, Hospital Huriez, CHRU, Lille Cedex, France.
Marc
M
Lambert
David
D
Launay
Eric
E
Hachulla
Sandrine
S
Morell-Dubois
Vincent
V
Soland
Viviane
V
Queyrel
François
F
Fourrier
Pierre-Yves
PY
Hatron
D000328:Adult; D019559:Capillary Leak Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D015430:Weight Gain
D016756:Immunoglobulins, Intravenous