Catastrophic antiphospholipid syndrome

Catastrophic antiphospholipid syndrome or Asherson syndrome is a rare but serious form of antiphospholipid syndrome which can develop in a patient with the classic form of the disease. It presents as multiple organ thromboses, evidence of organ dysfunction and thrombotic microangiopathy developing in less than a week.

Epidemiology

Less than 1% of patients with classic antiphospholipid syndrome will go on to develop catastrophic antiphospholipid syndrome .

Clinical presentation

Patients most commonly present with renal (e.g. renal vein thrombosis), pulmonary (e.g. pulmonary embolism) or central nervous system (e.g. ischemic stroke) involvement although any organ system can be affected .

Pathology

The underlying pathogenesis is similar to classic antiphospholipid syndrome but the severity of the disease is more severe . Due to the extent of the organ involvement a systemic inflammatory response syndrome is usually present also. Precipitating factors may include infection, medication or surgery.

Catastrophic antiphospholipid syndrome is characterized by :

• evidence of involvement of three or more organ systems
• development of manifestations simultaneously or in less than a week
• confirmation of small-vessel occlusion on biopsy
• laboratory confirmation of the presence of antiphospholipid antibodies

Radiographic features

The features of catastrophic antiphospholipid syndrome are identical to the manifestations of classic antiphospholipid syndrome except that multiple organ systems are involved acutely and concurrently.

Treatment and prognosis

Anticoagulation, steroids, plasma exchange, cyclophosphomide and intravenous immunoglobulin therapy are often used . The longterm mortality of catastrophic antiphospholipid syndrome is nearly 50%. Patients may develop further events of catastrophic antiphospholipid syndrome after the initial one.