Catastrophic Antiphospholipid Antibody Syndrome (Thrombotic Storm)

Catastrophic Antiphospholipid Syndrome is a very rare complication encountered in a subset of patients with antiphospholipid antibody syndrome. This rare syndrome that is characterized by the development of multiple blood clots that block small blood vessels in several organs in the body. The organs most commonly affected by these small blood clots include the heart, lungs, nervous system, and kidneys. In many ways, this syndrome is similar to another rare disease, thrombotic thrombocytopenic purpura.

Many patients who develop this rare complication have lupus, and infections have been reported to potentially increase a patient's risk to develop the syndrome. Even with the best treatment, as many as half the patients who develop this syndrome do not survive.

Treatment includes anticoagulation (blood thinners), steroids, and a procedure called 'plasma exchange'. Plasma exchange refers to a process whereby a patient's plasma (the liquid part of the blood) is removed and replaced with plasma from blood donors. Patients who survive this life-threatening complication are generally maintained on long-term anticoagulant therapy.

Thrombotic storm is characterized by the rapid development of multiple thromboses (blood clots) in a patient with an underlying hyper coagulable state. The catastrophic Antiphospholipid Syndrome is one type of thrombotic storm, but patients may have other risk factors for forming blood clots. Treatment is similar to the approach used for patients with catastrophic antiphospholipid syndrome.