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Lupus
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Susac’s syndrome or catastrophic antiphospholipid syndrome?

S Bucciarelli

Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain

R Cervera

Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain, rcervera{at}clinic.ub.es

M Martínez

Department of Neurology, Hospital Verge de Meritxell, Andorra la Vella, Andorra

X Latorre

Department of Internal Medicine, Hospital Verge de Meritxell, Andorra la Vella, Andorra

J Font

Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain

Susac’s syndrome is a microangiopathic disorder of unknown pathogenesis presenting with encephalopathy, hearing loss and branch retinal artery occlusions. The term ‘catastrophic’ antiphospholipid syndrome (APS) is used to define a subset of the APS characterized by thrombotic microangiopathy with clinical evidence of three or more organ involvement developed in a short period of time. We describe a patient with typical features of Susac’s syndrome, that appeared in less than a week, in whom aPL were detected, thus fulfilling criteria for ‘probable’ catastrophic APS.

Key Words: antiphospholipid syndrome • catastrophic antiphospholipid syndrome • Susac’s syndrome • thrombosis

Lupus, Vol. 13, No. 8, 607-608 (2004)
DOI: 10.1191/0961203304lu1046cr
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