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[Systemic capillary leak syndrome].

E Beuls1, B J Rijnders

  • 1Universitaire Ziekenhuizen, afd. Algemeen Interne Geneeskunde, Leuven, België.

Nederlands Tijdschrift Voor Geneeskunde
|February 22, 2000
PubMed
Summary

Systemic capillary leak syndrome (SCLS) is a rare, fatal condition causing plasma leakage. Early recognition and supportive therapies, including fluids and specific medications, are crucial for managing shock episodes.

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Area of Science:

  • Internal Medicine
  • Hematology
  • Nephrology

Background:

  • Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent episodes of plasma extravasation.
  • Its etiology and pathogenesis remain largely unknown, posing diagnostic and therapeutic challenges.

Observation:

  • A 61-year-old male presented with recurrent shock episodes preceded by prodromal symptoms like rhinorrhea, epigastric discomfort, nausea, vertigo, orthostatism, and low-grade fever.
  • During shock, marked hypotension, hemoconcentration (elevated hematocrit), hypoalbuminemia, and prerenal kidney failure were observed.
  • The patient also had an IgG-kappa paraprotein, and shock episodes responded rapidly to intravenous fluid resuscitation, followed by polyuria.

Findings:

  • The clinical presentation and laboratory findings were characteristic of systemic capillary leak syndrome.
  • The presence of an IgG-kappa paraprotein may be associated with SCLS in some cases.
  • Rapid fluid resuscitation effectively managed hypotensive episodes, and polyuria indicated fluid mobilization post-attack.

Implications:

  • SCLS requires consideration in the differential diagnosis of unexplained shock, including idiopathic and anaphylactic shock.
  • Supportive care, including fluid administration and inotropics, is vital for acute management.
  • Terbutaline and theophylline show promise in preventing future SCLS attacks, warranting further investigation.

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