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Additional triggers of endothelial disfunction modulate antiphospholipid-mediated microangiopathy in a central nervous system-cutaneous syndrome


 

CER675
2010 Vol.28, N°3
PI 0411, PF 0415
Case Reports

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PMID: 20525448 [PubMed]

Received: 23/10/2009
Accepted : 19/01/2010
In Press: 23/06/2010
Published: 23/06/2010

Abstract

It is now recognised that the spectrum of antiphospholipid (aPL)-mediated syndromes includes end-organ injury due to microangiopathic manifestations. In the central nervous system (CNS), the clinical and radiographic appearance of microangiopathic lesions can be notoriously difficult to distinguish from multiple sclerosis (MS). A patient is presented who developed white-matter lesions in the brain and spinal-cord, shortly after receiving toxic doses of radiation for an arterio-venous malformation. The institution of interferon therapy for presumptive MS not only led to worsening neurologic deficits, but triggered a cutaneous syndrome with pleomorphic stigmata of microvascular injury (livedo reticularis rash, splinter haemorrhages). Subsequent workup revealed persistently elevated high-titer antiphospholipid of multiple isotypes. Treatment with corticosteroids and immunosuppressant therapy afforded improvement in locomotor function. We hypothesise that radiation injury and treatment with interferon-therapy constituted iatrogenic `hits` of endothelial injury, and potentiated aPL-mediated microangiopathic disease affecting the CNS and the skin.

Rheumatology Article