35. To kill or cure? The diagnostic challenge in a patient with cerebral lupus

Mark D J Gibson

doi:10.1093/rap/rky033.026

Abstract

35. To kill or cure? The diagnostic challenge in a patient with cerebral lupus

Highlights

Lupus patients presenting with neurology symptoms continues to represent a diagnostic challenge for clinicians as the neurology may be attributable manifestation of their disease or as a result of immunosuppression
Cerebral lupus vasculitis is a rare manifestation of systemic lupus erythematosus (SLE) (7-12% SLE post mortem studies) and is associated with a high mortality (67%)
Key Learning Points: A florid necrotising cerebral lupus vasculitis can occur despite normal anti-dsDNA titres and complement levels

Summary

Introduction

Sneddon’s syndrome, first described in 1965 is a rare clinical syndrome of multiple ischaemic strokes, neurological symptoms and livedo racemosa. In light of the haematuria, she underwent a renal biopsy and was found to have IgA nephropathy consistent with a diagnosis of Henoch Schonlein purpura Her other co-morbidities included a previous ventricular septal defect, repaired in childhood and recurrent migraines. An MRI was arranged and revealed a recent cortical infarct in the left parieto-occipital region, with multiple previous cortical infarcts She was treated as a stroke secondary to possible vasculitis, in light of her younger age and initial MRI appearances. Key Learning Points: Sneddon’s syndrome is arare conditionof multiple strokes and livedo racemosa It is often associated with antiphospholipid antibodies and may be seen in anti-phospholipid syndrome.

36. RECURRENT STROKES IN YOUNGER PATIENT WITH RASH: A CASE OF SNEDDON’S SYNDROME