Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature.
The systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic attacks of systemic capillary hyperpermeability accompanied by hypoalbuminemia, hemoconcentration and edema. Treatment of the acute phase is supportive, focusing on adequate fluid resuscitation. Many agents have been used to prevent acute attacks, including corticosteroids, β2-agonists (aminophylline, theophylline, or terbutaline), infliximab, thalidomide and intravenous immunoglobulin (IVIg). β2-agonists were the first-line maintenance therapy until a few years ago. In more recent years, IVIg became common first-line prophylactic therapy in most patients with benefits at the dose of 2 g/kg once a month. We report the case of a 49-year-old man with SCLS treated successfully with a lower dose of IVIg (1 g/kg monthly) in the maintenance phase. He presented no acute episodes in a follow-up of 28 months. We describe prophylactic treatments for SCLS in literature and compare our patient to another 18 who received IVIg in follow-up.
Department of Clinical Medicine, Sapienza University of Rome, Clinical Immunology Unit, Rome, Italy.
Alessandro M
AM
Marra
Antonietta
A
Gigante
Edoardo
E
Rosato
D019559:Capillary Leak Syndrome / Q000276:immunology / Q000628:therapy*; D002199:Capillary Permeability / Q000187:drug effects; D002986:Clinical Trials as Topic; D018572:Disease-Free Survival; D004487:Edema / Q000209:etiology / Q000517:prevention & control*; D005060:Europe; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012769:Shock / Q000209:etiology / Q000517:prevention & control*; D013726:Terbutaline / Q000008:administration & dosage; D013806:Theophylline / Q000008:administration & dosage
D016756:Immunoglobulins, Intravenous; D013806:Theophylline; D013726:Terbutaline