systemic capillary leak syndrome

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)

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

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
acute kidney injury (AKI) / acute renal failure (ARF) may occur
rhabdomyolysis may occur from oedema causing compartment syndromes
lab features:
- raised haematocrit due to plasma loss
- hypoalbuminaemia
- a paraprotein in the blood (an monoclonal gammopathy of uncertain significance (MGUS) in approximately 80% of cases)

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
corticosteroids