An Unusual Presentation of Takayasu Arteritis: Bilateral Renal Artery Aneurysm

Introduction: Takayasu arteritis (TA) is a rare large-vessel vasculitis of unknown etiology, affecting mainly young women. The onset of symptoms is mostly subacute, leading to a possible delay in diagnosis.

Case Presentation: We describe a case of TA in a 14 year-old Caucasian female with negative past medical history complicated by the rupture of a right renal artery aneurysm and leading to impaired kidney function.

At admission, she presented acute severe abdominal pain and hypovolemic shock. The blood tests indicated severe anemia, mildly elevated inflammation markers and acute kidney injury. Abdominal imaging studies confirmed the active bleeding from the ruptured aneurysm, requiring surgical hemostasis, right nephrectomy and temporary hemodialysis. A second aneurysm with thrombotic signs affecting the left renal artery, partially supplying the ipsilateral kidney, was revealed. The histological findings of the removed tissue were consistent with a partially calcified older aneurysm, with thrombi of different histological ages and dystrophic changes in the elastic lamina. A prothrombotic state was also discovered.

While receiving hemodialysis a CT Angiography was performed, showing multiple arterial aneurysms affecting the intracranial segment of the left internal carotid artery, the abdominal aorta, and the mesenteric arteries. Correlating the data, late-stage TA was the favoured diagnosis. The renal involvement led to the development of stage 3 chronic kidney disease, with severe cardiovascular consequences (hypertension (HT) and hypertrophic cardiomyopathy).

The main challenge lay in preserving the kidney function and reducing its cardiovascular effects, thus hindering the usage of contrast-enhanced imaging techniques in follow-ups.

Discussion: There is little evidence regarding therapy in late-phase TA. There are studies suggesting the presence of complications such as aneurysms and HT may lead to a poorer outcome. The additional renal damage and thrombophilic disorder had an important impact in terms of management and treatment, requiring a multidisciplinary approach and close follow-up.