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scls

systemic capillary leak syndrome

see also:

Introduction

  • a rare episodic disorder characterised but increased permeability of capillary walls and loss of plasma protein into the tissues which may be life threatening
  • no clear definite hereditary pattern
  • mainly affects healthy middle aged people
  • aka Clarkson syndrome
  • rarely may be precipitated by Astra-Zeneca Covid-19 vaccine (less than 1 in 10 million doses)

Two phases

capillary leak phase

  • lasts 1-3 days during which up to 70% of plasma may leak into the tissues

recruitment phase

  • reabsorption of the initially extravasated fluid and albumin from the tissues, and it usually lasts 1 to 2 days
  • this may cause intravascular fluid overload leading to polyuria and can cause flash pulmonary edema and cardiac arrest

Clinical features of acute phase

  • may initially have URTI or gastro-like symptoms or limb aches before developing thirst, light-headedness as a result of reduced blood volume and hypotension (systolic BP < 90mmHg)
  • systemic or peripheral oedema usually is present often with cold peripheries
  • rhabdomyolysis may occur from oedema causing compartment syndromes
  • lab features:

Treatment

  • generally require close monitoring in ICU watching for compartment syndrome and APO which will require usual Mx
  • support BP with albumin but this generally is transient effect and creates more oedema

Prevention of further episodes

  • IVIG seems to provide the best long term outcomes
scls.txt · Last modified: 2021/06/13 10:28 by gary1