An Uncommon Presentation of Takayasu Arteritis: A Diagnostic Challenge with Posterior Reversible Encephalopathy Syndrome

Abstract

Takayasu arteritis (TA), a large-vessel vasculitis affecting young females more, may present with diverse clinical manifestations. This case report describes an 11-year-old girl who presented with headache, vomiting, bilateral ocular deviation, and altered sensorium. Clinical examination revealed hypertensive emergency and grade IV papilledema. Brain imaging demonstrated white-matter edema in the occipital and parietal lobes, suggestive of Posterior reversible encephalopathy syndrome (PRES). Color Doppler of abdominal vessels and computed tomography angiography revealed left renal artery stenosis, leading to the diagnosis of TA as per European League Against Rheumatism/ Pediatric Rheumatology International Trial Organization consensus criteria. This case highlights the rare association between TA and PRES, emphasizing the importance of considering underlying vasculitis in children presenting with hypertensive emergencies and neurological symptoms. The patient was managed with antihypertensive and immunosuppressive agents, including corticosteroids and mycophenolate mofetil, resulting in symptomatic improvement. Surgical intervention was done for definitive management. This report underscores the significance of early recognition and treatment of PRES in the context of TA to prevent potentially catastrophic consequences and establish causality.

Cite this article as: Prasad R, Gupta A, Singh A, et al. An uncommon presentation of Takayasu arteritis: A diagnostic challenge with posterior reversible encephalopathy syndrome. Eur J Rheumatol. 2025, 12(3), 0002, doi: 10.5152/eurjrheum.2025.24002.