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

Clinical and Experimental Rheumatology

Julius Birnbaum¹ 

关键词: Antiphospholipid antibody syndrome;    neuropsychiatric disease;   

DOI  :  

学科分类：医学（综合）

来源: Pacini Editore SpA

【 摘 要 】

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.

【 授权许可】

Unknown