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

[systemic capillary leak syndrome]

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

Diseases
Validation

Diseases presenting "acute phase" symptom

allergic bronchopulmonary aspergillosis

benign recurrent intrahepatic cholestasis

focal myositis

hydrocephalus with stenosis of the aqueduct of sylvius

locked-in syndrome

neuralgic amyotrophy

scrub typhus

severe combined immunodeficiency

systemic capillary leak syndrome

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