Idiopathic systemic capillary leak syndrome (ISCLS)

Idiopathic systemic capillary leak syndrome (SCLS), or Clarkson’s disease was first described by Clarkson in 1960.

• is an unusual and life-threatening disease caused by unexplained systemic capillary striking hyperpermeability, and the classic acute SCLS can be described as a triad of “3 Hs”, namely, hypotension, hypoalbuminaemia and haemoconcentration
  • characterized by stereotypic “attacks”of varying intensity of hypovolemic shock and generalized oedema in association with haemoconcentration (as detected by an elevated haematocrit concentration) and hypoalbuminaemia, typically occurring in the absence of albuminuria (1)
  • during the recovery phase, extravasated fluids are recruited back into the intravascular space
    • during this period, the patient is at high risk for intravascular volume overload and pulmonary oedema
• cause of ISCLS is not known although there is an associated monoclonal gammopathy in many patients
  • presence of serum M protein, a notable hallmark, is the only pertinent laboratory abnormality during the quiescent phase between the attacks (1)
• ISCLS is a type of distributive shock complicated by systemic capillary leak
  • therefore other possible causes such as sepsis, anaphylaxis, and certain drug reactions need to be excluded
• complications associated with ISCLS include (2):
  • renal failure,
  • stroke,
  • deep-vein thrombosis,
  • compartment syndrome,
  • rhabdomyolysis, and
  • cardiopulmonary failure

Reference:

1. Kapoor P, Greipp PT, Schaefer EW, Mandrekar SJ, Kamal AH, Gonzalez-Paz NC, et al. Idiopathic systemic capillary leak syndrome (Clarkson's disease): The mayo clinic experience. Mayo Clin Proc. 2010;85:905–12.
2. Hajare KR, Patil P, Bansode J. Idiopathic Systemic Capillary Leak Syndrome. Indian J Crit Care Med. 2018 May;22(5):369-371.