Catastrophic antiphospholipid syndrome

Catastrophic antiphospholipid syndrome, CAPS, also 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 failure, and tissue necrosis and is considered an extreme variant of the antiphospholipid syndrome.

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CAPS has a mortality rate of about 50 %. With the establishment of a CAPS-Registry more has been learned about this syndrome, yet the cause remains elusive. Infection, trauma, medication, 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. Patients exhibit a positive test to antilipid antibodies, typically IgG, and often have a history of lupus or another connective tissue disease. Thrombocytopenia is a common finding.

Clinically, the syndrome can affect many organs systems. Peripheral thrombosis may be encountered affecting veins or arteries. Intraabdominal thrombosis may lead to pain. Renal and pulmonary complications are common. The patient may exhibit skin purpura and necrosis. Cerebral manifestations may lead to encephalopathy and seizures. Myocardial infarctions may occur. Death may result from multiple organ failure.

Treatments may involve the following steps:

• Prevention includes the use of antibiotics for infection and parenteral anticoagulation for susceptible patients.
• Specific therapy includes the use of intravenous heparin, corticosteroids, and possibly plasma exchanges, intravenous immunoglobulin.
• Additional steps may have to be taken to manage circulatory problems, renal failure, and respiratory distress.

Note

This Asherson’s syndrome is not to be confused with the Asherson’s syndrome that is named after Nehemiah Asherson [1]

References

• 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. [2]