Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome, understood.

Catastrophic antiphospholipid syndrome or CAPS is a variant of antiphospholipid syndrome which is sometimes known as Asherson's syndrome, after Ronald Asherson, the researcher who first described it.

The condition involves the occlusion or blockage of blood vessels in various organs throughout the body. These can include the bowels, brain, heart, kidney, lungs and skin. Catastrophic antiphospholipid syndrome manifests itself in multiple organ thrombosis, thrombotic microangiopathy, and tissue necrosis.

Peripheral thrombosis as part of catastrophic antiphospholipid syndrome can affect arteries and veins, and intraabdominal thrombosis may cause pain.

Complications with the cardiovascular and nervous systems, as well as the kidneys and lungs, are typically a result of catastrophic antiphospholipid syndrome. Patients affected can show signs of skin purpura. The arterial clotting involved can cause strokes, while cerebral manifestations can lead to seizures. The multiple organ failure precipitated by catastrophic antiphospholipid syndrome can lead to death.

Causes

The cause of catastrophic antiphospholipid syndrome is not yet known, but it does have recognized 'triggers', including surgery, trauma, infection and medication. It is understood that the activation of cytokines in the form of a cytokine storm can lead to organ failure.

It is also typical for catastrophic antiphospholipid syndrome patients to test positive for antilipid antibodies such as IgG, with the possibility of a history of connective tissue disease such as lupus.

Associative diseases might be classed as a 'secondary' antiphospholipid syndrome if they do not meet all the catastrophic antiphospholipid syndrome criteria.

Prevention

Catastrophic antiphospholipid syndrome can be prevented in susceptible patients through the use of antibiotics to fight parenteral anticoagulation and infection.

Treatment

Treatment for catastrophic antiphospholipid syndrome can include therapy which uses corticosteriods and intravenous heparin, as well as intravenous immunoglobulin. Treatment is typically administered to directly address circulatory or respiratory problems which can be a feature of catastrophic antiphospholipid syndrome. Patients are also given Rituxan, which helps them retain stability when suffering from the disease.

Plasma exchange is a procedure which is used for patients with catastrophic antiphospholipid syndrome. It involves the removal of the patient's liquid blood (plasma) and replacing with the blood from blood donors.

A patient's history of thrombotic events can be a big factor in which treatment he or she will be administered. It should be noted that patients with the syndrome account for less than 1% of all patients with antiphospholipid syndrome.

Prognosis

The mortality rate of catastrophic antiphospholipid syndrome stands at around 50%. The success of treatment often comes down to the nature of the manifestations and their intensity. Earlier treatment is accepted to produce better outcomes.

Patients who do survive this life-threatening disease usually keep receiving anticoagulant therapy over the long term.

CAPS Registry

Management of the syndrome has been helped by the formation of the CAPS Registry, which was founded in 2000 as an international forum for patients with the disease. It was created by the European Forum on APL.

It allows all the published case reports of catastrophic antiphospholipid syndrome from around the globe to be correlated, including cases which have been newly diagnosed. Its creation is especially important for advances in the understanding of a rare disease such as catastrophic antiphospholipid syndrome, which can be hard to study because of the lack of information upon which to base research.

So far, the data of some 400 patients is available to view on the web. This resource covers laboratory, clinical and therapeutic information which has helped greatly when it comes to the characterization of catastrophic antiphospholipid syndrome's features.

The initial criteria needed to classify it are now more established, as are the guidelines of managing the condition. A Journal of Autoimmunity study is one of the most comprehensive conducted using data from the CAPS Registry. It looked at the cases of 280 catastrophic antiphospholipid syndrome patients.

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Last Reviewed:
August 29, 2017
Last Updated:
October 06, 2017
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