Catastrophic antiphospholipid antibody syndrome (CAPS)

Classification criteria for catastrophic antiphospholipid antibody syndrome (CAPS)

Criteria

1. Evidence of involvement of three or more organs, systems, and/or tissues

2. Development of manifestations simultaneously or in less than a week

3. Confirmation by histopathology of small vessel occlusion in at least one organ or tissue

4. Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibodies, and/or anti-beta2-glycoprotein I antibodies)

Classification

Definite catastrophic APS

Requires all four criteria

Probable catastrophic APS

All four criteria, except for only two organs, systems, and/or sites of tissue involvement or

All four criteria, except for the laboratory confirmation at least six weeks apart due to the early death of a patient never tested for aPL before the catastrophic APS or

Criteria 1, 2, and 4 above or

1, 3, and 4 and the development of a third event in more than a week but less than a month, despite anticoagulation

aPL: antiphospholipid antibodies.

Adapted from: Asherson, RA, Cervera, R, de Groot, PG, et al. Lupus 2003; 12:530.