Evaluation and diagnosis of catastrophic antiphospholipid syndrome (CAPS)

CAPS diagnosis requires clinical judgment and flexibility in interpretation; involvement of a hemostasis/thrombosis or CAPS expert is advised. Some individuals use classification criteria (described in UpToDate) for diagnosis, but these criteria were designed for research and should not replace clinical judgment. A high suspicion for other diagnoses should be maintained, as these other conditions can cause MAHA and thrombocytopenia and can accompany, trigger, or worsen CAPS or APS. Given its high mortality, empiric treatment for presumed CAPS is usually justified prior to diagnostic confirmation.

CAPS: catastrophic antiphospholipid syndrome; aPL: antiphospholipid antibodies; APS: antiphospholipid syndrome; DIC: disseminated intravascular coagulation; PT: prothrombin time; aPTT: activated partial thromboplastin time; HIT: heparin-induced thrombocytopenia; VITT: COVID-19 vaccine-included immune thrombotic thrombocytopenia; ELISA: enzyme-linked immunosorbent assay; TTP: thrombotic thrombocytopenic purpura; CM-TMA: complement-mediated thrombotic microangiopathy, also called atypical HUS; HUS: hemolytic uremic syndrome; STEC: Shiga toxin producing Escherichia coli; HELLP: hemolysis, elevated liver function tests, and low platelets; MAHA: microangiopathic hemolytic anemia.

* aPL include anticardiolipin and anti-beta2-glycoproteinI antibodies, assayed by ELISA, and lupus anticoagulant, detected by clotting assay. Persistent positivity refers to two positive tests ≥12 weeks apart. UpToDate discussions of APS provide information on clinical relevance.

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Evaluation and diagnosis of catastrophic antiphospholipid syndrome (CAPS)