Systemic capillary leak syndrome (SCLS)

Version: 2.1
Published: 11 February 2016
Condition for which Ig use is in exceptional circumstances only

This medical condition has either been superseded or has become inactive
View Current Version  Print
Specific Conditions
  • Systemic capillary leak syndrome
Level of Evidence Insufficient data (Category 4a)
SCLS is an extremely rare condition that is characterised by life-threatening attacks of reversible capillary hyperpermeability, accompanied by haemoconcentration and hypoalbuminaemia.

A diagnosis by a consultant physician, emergency specialist or intensive care unit specialist is required.

Other therapies may be appropriate.

Approval will be provided for an initial period of 12 months only.

Clinicians requesting ongoing intravenous immunoglobulin (IVIg) therapy after the initial 12-month period are required to confirm in writing that the patient experienced a reduced number of severe episodes requiring hospital admission when treated with IVIg.

Maximum dose of 1–2 g/kg per month.

Refer to the current product information sheet for further information.

The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
 
Bibliography
Abueguen, P, Chennebault, JM & Pichard, E 2010, ‘Immunoglobulins for the treatment of systemic capillary leak syndrome’, Americal Journal of Medicine, vol. 123, pp. e3–4.

Druey, KM & Greipp, PR 2010, ‘Narrative review: the systemic capillary leak syndrome’, Annals of Internal Medcine, vol. 153, pp. 90–8.

Gousseff, M, Arnaud, L, Lambert, M, et al 2011, ‘The systemic capillary leak syndrome: a case series of 28 patients from a European registry’, Annals of Internal Medicine, vol. 154, pp. 464–71.

Govig, BA & Javaheri, S 2010, ‘The systemic capillary leak syndrome (letter)’, Annals of Internal Medicine, vol. 153, p. 764.

Lambert, M, Launay, D, Hachulla, E, et al 2008, ‘High-dose intravenous immunoglobulins dramatically reverse systemic capillary leak syndrome’, Critical Care Medicine, vol. 36, pp. 2184–7.

Zipponi, M, Eugster, R & Birrenbach, T 2011, ‘High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome’, BMJ Case Reports, doi:10.1136/bcr.12.2010.3599.