Disseminated Takayasu arteritis with neurovascular small and medium vessel involvement

摘要

Takayasu arteritis is a rare granulomatous vasculitis that is commonly classified as a large vessel disease. Small and medium vessel involvement are extremely rare, with only a few case reports describing neurovascular, ocular and cutaneous involvement. We describe a 21 year old Malaysian woman with pre-existing Takayasu arteritis confined to the large vessels, presenting one year later to our centre with status epilepticus. Extensive radiologic studies revealed diffuse encephalopathic changes and multifocal neurovascular involvement, with the vasculitis progressing to encompass the large, medium and small vessels. The patient was treated with high dose steroid therapy and discharged well with long term steroid and immunosuppressive therapy. Follow up MRI with vessel wall imaging sequence (VW-MR) and arterial spin labelling (ASL) perfusion imaging demonstrated intra and extracranial vessel wall inflammation, with generalized reduction in left sided cerebral blood flow. This case demonstrates that Takayasu arteritis is not exclusively a large vessel vasculitis, and that small and medium vessel involvement does not preclude its diagnosis.