scls
Table of Contents
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:
- raised haematocrit due to plasma loss
- a paraprotein in the blood (an monoclonal gammopathy of uncertain significance (MGUS) in approximately 80% of cases)
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 00:28 by gary1