Table 1.

Validated classification criteria for definitive CAPS.

1.Evidence of involvement of 3 or more organs. 2.Development of manifestations simultaneously or within less than a week. 3.Confirmation by histopathology of small vessel occlusion. 4.Laboratory confirmation of the presence of elevated titres of antiphospholipid antibodies (>12 weeks). Probable CAPS if only three out of the four criteria above are met.