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Livedo aPL Significance — SCE Rheumatology MCQ

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ModerateSLE & Antiphospholipid SyndromeLivedo aPL SignificanceSCE Rheumatology

A 55-year-old woman with SLE has persistent, irregular, broken, branching livedo over her trunk and limbs that does not disappear on warming. Lupus anticoagulant and high-titre IgG anticardiolipin antibodies remain positive on repeat testing 12 weeks apart. She has had no thrombosis, pregnancy morbidity or neurological symptoms. Which is the best interpretation and management approach?

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Correct answer: E — An aPL-associated non-criteria vasculopathy; assess thrombotic risk and consider aspirin

The persistent, irregular pattern is livedo racemosa, an aPL-associated, non-criteria cutaneous small-vessel manifestation rather than physiological cold-induced livedo. It should prompt assessment of the full thrombotic risk profile and control of modifiable cardiovascular and venous-thromboembolism risks. This patient has a high-risk aPL profile because lupus anticoagulant and high-titre anticardiolipin antibodies are persistently present. EULAR recommends considering low-dose aspirin, usually 75–100 mg daily, in SLE without previous thrombosis but with a high-risk aPL profile, after considering bleeding risk. Livedo alone does not establish thrombotic APS or Sneddon syndrome; Sneddon syndrome requires associated cerebrovascular disease. There is no evidence here of inflammatory cutaneous vasculitis or other organ-threatening lupus warranting high-dose glucocorticoids or cyclophosphamide, and therapeutic anticoagulation is not indicated without a thrombotic event.

Reference: EULAR, Fanouriakis A et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update, recommendation 12, published online 12 October 2023. https://www.eular.org/document/download/724/fe9e2ce7-b682-4f2f-aa41-7983eeb7a05c/697