High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome.

Abstract

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.

DESIGN:

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.

MAIN 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.

• Authors: Marc Lambert, David Launay, Eric Hachulla, Sandrine Morell-Dubois, Vincent Soland, Viviane Queyrel, François Fourrier, Pierre-Yves Hatron
• Journal: Critical care medicine (Crit Care Med) Vol. 36 Issue 7 Pg. 2184-7 (Jul 2008) ISSN: 1530-0293 [Electronic] United States
• PMID: 18552679 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulins, Intravenous

Topics

• Adult
• Capillary Leak Syndrome (diagnosis, drug therapy, physiopathology)
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Male
• Weight Gain