Asherson et al listed criteria for the diagnosis of the catastrophic antiphospholipid syndrome (CAPS).
Criteria:
(1) involvement of organs, organ systems or tissues
(2) rapid progression (simultaneously or in less than 1 week)
(3) laboratory demonstration of antiphospholipid antibodies
(4) histologic evidence of small vessel occlusion (may involve 1 or more sites; may be accompanied by vasculitis)
Number of Organs |
Progression |
Lab Evidence |
Histologic Evidence |
Diagnosis |
>= 3 |
acute |
present |
present |
definite |
2 |
acute |
present |
present |
probable |
>= 3 |
abute |
not available |
present |
probable |
>= 3 |
subacute |
present |
present |
probable |
where:
• Involvement of an organ or tissue may be demonstrated by imaging studies.
• Renal involvement is defined by an increase in serum creatinine by 50% or more, severe arterial hypertension and/or proteinuria.
• Subacute progression may involve progression from 1 to 4 weeks after presentation despite anticoagulation.
• Laboratory evidence may not available if testing was not done or if the patient dies.
Specialty: Hematology Oncology, Clinical Laboratory, Immunology/Rheumatology