Abstract

Seronegative antiphospholipid syndrome (seronegative APS) has remained an enigma and the concept is controversial. A small group of APS patients remain persistently negative for routine assays of antiphospholipid (aPL) antibodies. The clinical features are well defined, and include the tendency to both arterial and venous thrombosis, to recurrent miscarriages, and to occasional thrombocytopenia. Patients with clinical manifestations highly suggestive of APS but persistently negative conventional aPLs are classified as having seronegative APS.

Highlights

  • Seronegative antiphospholipid syndrome has remained an enigma and the concept is controversial

  • The number of patients with antiphospholipid syndrome (APS) grows, it comes as no surprise that seronegative APS provides the focus of day to day clinical discussion – the patient with migraine, stroke, several previous miscarriages, thrombocytopenia, and livedo reticularis, whose aPL tests are doggedly negative

  • Livedo reticularis shares a number of features with APS such as pregnancy loss, arterial thrombosis, heart valve abnormalities and seizures and it is the most common cutaneous manifestation of APS

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Summary

Introduction

Seronegative antiphospholipid syndrome (seronegative APS) has remained an enigma and the concept is controversial. The antiphospholipid syndrome is an autoimmune disease characterised by arterial and/or venous thromboses, recurrent abortions or foetal loss, and circulating antiphospholipid (aPL) antibodies [1,2,3,4,5]. It is universally recognized that the routine screening tests - the anti-cardiolipin (aCL) antibodies and lupus anticoagulant, may miss some cases. A small group of APS patients remain persistently negative for routine assays of aPL [3,4,5,6,7,8].

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