Classification criteria for catastrophic antiphospholipid antibody syndrome (CAPS)

Criteria

Evidence of involvement of 3 or more organs, systems, and/or tissues

Development of manifestations simultaneously or in less than a week

Confirmation by histopathology of small vessel occlusion in at least 1 organ or tissue

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 4 criteria

Probable catastrophic APS

All 4 criteria, except for only two organs, systems, and/or sites of tissue involvement or

All 4 criteria, except for the laboratory confirmation at least 6 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.

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Classification criteria for catastrophic antiphospholipid antibody syndrome (CAPS)