Catastrophic antiphospholipid syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening cause of multi-organ failure with an acute onset (1).

• observed in less than 1% of APS patients (2)
• it is characterized by the development of microthrombi in multiple small vascular beds in several organ systems leading to multioragn failure e.g. - typically the brain, kidneys, lung and the skin

Important differential diagnosis include -thrombotic thrombocytopaenic purpura, haemolytic uraemic syndrome, and disseminated intravascular coagulation (2)

Combinations of treatment are typically used including anticoagulation with heparin/warfarin. In addition Immunomodulatory therapies including plasmaphaeresis, intravenous human IgG, corticosteroids and rituximab have been employed (1).

Reference:

• (1) Keeling D et al.Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol. 2012;157(1):47-58.