Management of catastrophic antiphospholipid syndrome (CAPS)

CAPS: catastrophic antiphospholipid syndrome; TPE: therapeutic plasma exchange; IVIG: intravenous immune globulin; aPL: antiphospholipid antibodies; LDH: lactate dehydrogenase; PT: prothrombin time; INR: international normalized ratio; SLE: systemic lupus erythematosus; LMW: low-molecular-weight; HIT: heparin-induced thrombocytopenia; IV: intravenous; TPE: therapeutic plasma exchange; TTP: thrombotic thrombocytopenic purpura; TMA: thrombotic microangiopathy; APS: antiphospholipid syndrome; ELISA: enzyme-linked immunosorbent assay.

* Bleeding is a spectrum, and clinical judgment is required. We define major bleeding as blood loss requiring transfusion, bleeding into a critical closed space (intracranial, compartment syndrome), or bleeding requiring an intervention (surgery, endoscopic treatments). All bleeding is important to the patient; however, individuals with minor bleeding can usually tolerate anticoagulation.

¶ aPL testing includes anticardiolipin and anti-beta2-glycoproteinI antibodies, assayed by ELISA, and lupus anticoagulant, assayed by a functional clotting assay.

Δ Rituximab is generally appropriate for presentations with significant microvascular thromboses. Eculizumab may be appropriate for presentations consistent with complement-mediated TMA.