Idiopathic systemic capillary leak syndrome (clarkson disease)

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Idiopathic systemic capillary leak syndrome (SCLS) or “Clarkson disease” is a life-threatening disorder characterized by microvascular hyperpermeability-related recurrent “attacks” or “flares” of severe hypotension, edema, hemoconcentration, and hypoalbuminemia. A monoclonal protein, detected in the majority of the patients, serves as a valuable clue for the diagnosis of SCLS, a disorder with myriad non-specific prodromal and acute attack-related symptoms. Prompt recognition and timely institution of appropriate supportive care are critical. Restoration of normal perfusion requires judicious use of vasopressors, fluids, and occasionally diuretics. Prophylactic therapy with beta-2 agonists, methylxanthines, or intravenous immunoglobulin may reduce the frequency and severity of “attacks.” Although in the recent years we have gained a better understanding of the biology of this perplexing disorder, there remains a paucity of high-level evidence, particularly with regard to efficacy of the currently available therapies. This chapter outlines the diagnostic dilemmas and current approaches related to the management of idiopathic SCLS.

Original languageEnglish (US)
Title of host publicationBiology and Management of Unusual Plasma Cell Dyscrasias
PublisherSpringer New York
Pages143-161
Number of pages19
ISBN (Electronic)9781441968487
ISBN (Print)9781441968470
DOIs
StatePublished - Jan 1 2016

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Keywords

  • Anasarca
  • Capillary hyperpermeability
  • Hemoconcentration
  • Monoclonal gammopathy
  • Shock

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Kapoor, P. (2016). Idiopathic systemic capillary leak syndrome (clarkson disease). In Biology and Management of Unusual Plasma Cell Dyscrasias (pp. 143-161). Springer New York. https://doi.org/10.1007/978-1-4419-6848-7_8