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Catastrophic antiphospholipid syndrome — SCE Rheumatology MCQ

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HardConnective tissue diseasesCatastrophic antiphospholipid syndromeSCE Rheumatology

A 45-year-old woman with known antiphospholipid syndrome is admitted with confusion, acute kidney injury, livedo and hypoxia. Platelets are 48 × 10^9/L, schistocytes are absent, CT chest shows pulmonary microthrombotic change and MRI brain shows multiple small infarcts. Blood cultures are pending and lupus anticoagulant is persistently positive. What is the most appropriate management?

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Correct answer: D — Anticoagulation, high-dose corticosteroids and plasma exchange or intravenous immunoglobulin

Rapid multiorgan thrombosis in a patient with APS suggests catastrophic APS, which requires urgent combination therapy, typically anticoagulation, high-dose steroids and plasma exchange or IVIG, while treating triggers such as infection. Warfarin alone is inadequate acutely and delayed anticoagulation risks further thrombosis. Sepsis may coexist but does not explain the full thrombotic syndrome.

Reference: EULAR APS recommendations; BSR SLE guideline