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CAPS Treatment — SCE Rheumatology MCQ

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HardSLE & Antiphospholipid SyndromeCAPS TreatmentSCE Rheumatology

A 55-year-old man with SLE develops catastrophic antiphospholipid syndrome (CAPS). He has rapid-onset multi-organ failure with renal failure encephalopathy ARDS and digital ischaemia over 3 days. aPL are strongly positive. What is the recommended treatment for CAPS?

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Correct answer: E — Triple therapy with anticoagulation (heparin) high-dose glucocorticoids AND plasma exchange or IVIg; Rituximab and Eculizumab may be considered for refractory cases

Catastrophic APS (CAPS) is defined by involvement of ≥3 organ systems within a week confirmed by biopsy showing small vessel occlusion and positive aPL. It carries mortality of approximately 30-50%. Treatment requires triple therapy: full anticoagulation (IV unfractionated heparin) high-dose glucocorticoids (IV Methylprednisolone 1 g daily for 3 days) AND plasma exchange (to remove aPL and cytokines) or IVIg (if plasma exchange is not available). For refractory cases Rituximab (targeting B cells producing aPL) and Eculizumab (complement inhibition) have been used with success. Precipitating factors (infection surgery medication withdrawal) should be identified and treated. The CAPS Registry provides ongoing outcome data.

Reference: Cervera R et al 2018 CAPS treatment; EULAR 2019 APS recommendations