A catastrophic seronegative anti-phospholipid syndrome: case and literature review

Thrombosis Journal

Table 1 Criteria for the classification of catastrophic APS⁵

1. Evidence of involvement of three or more organs, systems and/or tissues^a
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^b
4. Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies)^c
Definite catastrophic APS: All 4 criteria.
Probable catastrophic APS:
– All 4 criteria, except for only two organs, systems and/or tissues involvement.
– All 4 criteria, except for the absence of laboratory confirmation at least 6 weeks apart due to the early death of a patient never tested for aPL before the catastrophic APS.
– Criteria 1, 2 and 4.
– Criteria 1, 3 and 4 and the development of a third event in more than a week but less than a month, despite anticoagulation.

^a Usually, clinical evidence of vessel occlusions, confirmed by imaging techniques when appropriate. Renal involvement is defined by a 50% rise in serum creatinine, severe systemic hypertension (> 180/100 mmHg) and/or proteinuria (> 500 mg/24 h)
^b For histopathological confirmation, significant evidence of thrombosis must be present, although vasculitis may coexist occasionally
^c If the patient had not been previously diagnosed as having an APS, the laboratory confirmation requires that presence of antiphospholipid antibodies must be detected on two or more occasions at least 6 weeks apart (not necessarily at the time of the event), according to the proposed preliminary criteria for the classification of definite APS

ISSN: 1477-9560