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High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome

Lambert, Marc MD, PhD; Launay, David MD; Hachulla, Eric MD, PhD; Morell-Dubois, Sandrine MD; Soland, Vincent MD; Queyrel, Viviane MD; Fourrier, François MD; Hatron, Pierre-Yves MD

doi: 10.1097/CCM.0b013e31817d7c71
Case Report

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.

From the Department of Internal Medicine and Faculty of Medicine (ML, DL, EH, SM-D, VQ, P-YH), University of Lille II, Hospital Huriez, CHRU, Lille Cedex, France; the Intensive Care Unit and Faculty of Medicine (FF), University of Lille II, Hospital R.-Salengro CHRU, Lille Cedex, France; and the Intensive Care Unit (VS), CH Valenciennes, avenue Desandrouin, Valenciennes, France.

The authors have not disclosed any potential conflicts of interest.

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© 2008 by the Society of Critical Care Medicine and Lippincott Williams & Wilkins