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Fulminant leucocytoclastic brainstem vasculitis in a patient with otherwise indolent systemic lupus erythematosus

Department of Neurology, Royal Adelaide Hospital, Adelaide, SA, Australia; Department of Pathology, University of Adelaide, Adelaide, SA, Australia timothy.kleinig{at}adelaide.edu.au

B Koszyca

Department of Pathology, University of Adelaide, Adelaide, SA, Australia

PC Blumbergs

Department of Pathology, University of Adelaide, Adelaide, SA, Australia

P Thompson

Department of Neurology, Royal Adelaide Hospital, Adelaide, SA, Australia; Department of Medicine, University of Adelaide, Adelaide, SA, Australia

The spectrum of central nervous system (CNS) vascular pathology in systemic lupus erythematosus (SLE) includes small vessel vasculopathy, thromboembolism, perivascular lymphocytic infiltration and, rarely, overt transmural vasculitis. We present the case of a patient, who experienced three CNS relapses over total disease duration of 26 years, with otherwise indolent disease. The first two relapses were suspicious of vasculitis and the last was proven at autopsy. The short duration between final relapse onset and death in this SLE CNS vasculitis case was, to our knowledge, unique. Histopathological investigation demonstrated multiple confluent areas of haemorrhage in the medulla due to an acute small vessel leucocytoclastic vasculitis.

Key Words: cerebral vasculitis • intracerebral haemorrhage • systemic lupus erythematosus

Lupus, Vol. 18, No. 6, 486-490 (2009)
DOI: 10.1177/0961203308101548


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