Additional triggers of endothelial disfunction modulate antiphospholipid-mediated microangiopathy in a central nervous system-cutaneous syndrome.
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.
Division of Rheumatology, The John Hopkins University School of Medicine, Baltimore, USA. jbirnba2@jhmi.edu
Julius
J
Birnbaum
D016736:Antiphospholipid Syndrome / Q000150:complications / Q000276:immunology*; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D004730:Endothelium, Vascular / Q000276:immunology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D055806:Microvessels / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D015835:Ocular Motility Disorders / Q000209:etiology / Q000276:immunology / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology / Q000276:immunology*; D013116:Spinal Cord / Q000473:pathology; D014652:Vascular Diseases / Q000150:complications / Q000276:immunology*