Chronic systemic capillary leak syndrome treatment with intravenous immune globulin

Case report and review of the literature

Ryan Mullane, Eric Langewisch, Marius C Florescu, Troy J Plumb

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Systemic capillary leak syndrome (SCLS) is a disorder characterized by increased vascular permeability with intermittent acute episodes of profound capillary leak that may result in hypotension or shock. A rarely described chronic form of SCLS (cSCLS) presents as refractory edema, with pleural and/or pericardial effusions and hypoalbuminemia. These entities are differentiated by massive and periodic episodes of capillary leak, which can result in shock in SCLS, and chronic refractory edema in cSCLS. The etiologies of these disorders are poorly understood, but both acute and chronic forms often present with an associated monoclonal gammopathy. Flares of the SCLS have been reduced by treatment with intravenous immune globulin (IVIG). Only six cases of cSCLS have been reported, and previous treatments have included steroids, terbutaline, and theophylline. Based upon the reported responses of SCLS to IVIG, we present the case of a 54-year-old man with cSCLS where ongoing treatment with IVIG resulted in a marked and sustained improvement in the signs and symptoms of the capillary leak syndrome.

Original languageEnglish (US)
Pages (from-to)59-63
Number of pages5
JournalClinical Nephrology
Volume91
Issue number1
DOIs
StatePublished - Jan 1 2019

Fingerprint

Capillary Leak Syndrome
Intravenous Immunoglobulins
Shock
Edema
Therapeutics
Terbutaline
Hypoalbuminemia
Paraproteinemias
Pericardial Effusion
Capillary Permeability
Pleural Effusion
Theophylline
Hypotension
Signs and Symptoms
Steroids

Keywords

  • Chronic systemic capillary leak syndrome
  • Intravenous immunoglobulin

ASJC Scopus subject areas

  • Nephrology

Cite this

Chronic systemic capillary leak syndrome treatment with intravenous immune globulin : Case report and review of the literature. / Mullane, Ryan; Langewisch, Eric; Florescu, Marius C; Plumb, Troy J.

In: Clinical Nephrology, Vol. 91, No. 1, 01.01.2019, p. 59-63.

Research output: Contribution to journalArticle

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