Catastrophic antiphospholipid syndrome

From Wikipedia, the free encyclopedia
Jump to navigation Jump to search
Catastrophic antiphospholipid syndrome
Other namesCatastrophic APS

Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is an acute and complex biological process that leads to occlusion of small vessels of various organs. It was first described by Ronald Asherson in 1992. The syndrome exhibits thrombotic microangiopathy, multiple organ thrombosis, and in some cases tissue necrosis and is considered an extreme or catastrophic variant of the antiphospholipid syndrome.

CAPS has a mortality rate of about 50%. With the establishment of a CAPS-Registry more has been learned about this syndrome, but its cause remains unknown. Infection, trauma, medication, and/or surgery can be identified in about half the cases as a "trigger". It is thought that cytokines are activated leading to a cytokine storm with the potentially fatal consequences of organ failure. A low platelet count is a common finding.


Clinically, the syndrome affects at least three organs and may affect many organs systems. Peripheral thrombosis may be encountered affecting veins and arteries. Intra-abdominal thrombosis may lead to pain. Cardiovascular, nervous, kidney, and lung system complications are common. The affected individual may exhibit skin purpura and necrosis. Cerebral manifestations may lead to encephalopathy and seizures. Myocardial infarctions may occur. Strokes may occur due to the arterial clotting involvement. Death may result from multiple organ failure.


Individuals with CAPS often exhibit a positive test to antilipid antibodies, typically IgG, and may or may not have a history of lupus or another connective tissue disease. Association with another disease such as lupus is called a secondary APS unless it includes the defining criteria for CAPS.


Treatments may involve the following steps:


  • Walsh, N: "Clinical picture still emerging in CAPS registry". Ob.Gyn.News 2006; 41:25.
  • Asherson, RA. "The Catastrophic Antiphospholipid (Asherson’s) Syndrome". Autoimmunity Reviews 2005; 5:48–54. [1][dead link]
  • Hughes Syndrome foundation [2]
  • APS foundation of America, INC [3]
  1. ^ MD Consult Retrieved on 2009-06-02

External links[edit]

External resources