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Catastrophic antiphospholipid syndrome — SCE Acute Medicine MCQ

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HardAcute Rheumatological EmergenciesCatastrophic antiphospholipid syndromeSCE Acute Medicine

A woman with antiphospholipid syndrome develops pulmonary emboli, renal infarction, encephalopathy and thrombocytopenia within five days of stopping warfarin. Infection screening is positive for pyelonephritis. Which first-line treatment package best addresses catastrophic APS?

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Correct answer: A — Give therapeutic heparin, glucocorticoid and plasma exchange or IVIg

Explanation lettering: D = shown as A · C = shown as B · B = shown as C · E = shown as D · A = shown as E

D is correct. Rapid thrombosis in at least three organ systems strongly suggests catastrophic antiphospholipid syndrome. Recommended first-line “triple” treatment combines therapeutic anticoagulation—preferably heparin—with glucocorticoids and plasma exchange or intravenous immunoglobulin, while treating the precipitating infection. Warfarin alone acts too slowly, steroid alone leaves thrombosis untreated, and direct oral anticoagulants are unsuitable in high-risk APS. Rituximab or complement inhibition may be considered in refractory disease, not as routine initial monotherapy.

Reference: https://pmc.ncbi.nlm.nih.gov/articles/PMC11034817/