Inflammatory Pseudotumor of the Carotid Artery in a Patient With Takayasu’s Arteritis

Abstract

BACKGROUNDTakayasu's arteritis (TAK) is a large vessel vasculitis that mainly affects aorta and its primary branches. Inflammation causes thickening of the arteries walls, which may lead to stenosis, occlusion or dilation. However, inflammatory pseudotumor of the carotid artery has never been described in TAK. The aim of this report is to describe the case of a patient diagnosed with TAK evolving with a pulsatile, inflammatory neck mass. CASE REPORTWe herein report a case of a 31-year-old male patient, who presented with asymmetrical blood pressure and pulses in the upper limbs, left arm claudication, and elevated markers of inflammation. Computed tomography angiography (CTA) confirmed the diagnosis of type V TAK. He was initially treated with prednisone and azathioprine. However, azathioprine was discontinued due to gastrointestinal intolerance and was replaced by methotrexate. During follow-up, he evolved with disease flare (worsening of claudication and reduction in pulse of the left upper limb, elevation of inflammatory markers and worsening of left subclavian artery obstruction). A new induction therapy was prescribed with intravenous pulse therapy with methylprednisolone and cyclophosphamide, maintaining disease remission for 8 months. After that, a new disease flare occurred, and biological treatment was proposed. However, he had irregular medical follow-up and was unable to obtain the prescribed medications for several months. One month prior to the last medical visit, he evolved with clinical worsening and developed a fast-growing, pulsatile, right painful neck mass, associated with carotidynia, aphonia and systemic symptoms. He was referred to inpatient care for further investigation and respiratory monitoring. An urgent CTA was performed and revealed a large inflammatory vascular parietal thickening of the right carotid artery without evidence of aneurysmal formations, thrombosis or dissection, suggestive of inflammatory pseudotumor of the carotid; no evidence of external compression of upper airways was found. MRI showed the same findings of CTA. Videolaryngoscopy was performed and was normal, suggesting a psychogenic etiology to the aphonia. He was treated with pulse therapy with methylprednisolone and cyclophosphamide, and the mass promptly started to reduce. Biopsy was not feasible, since the mass almost disappeared within few days, due to the excellent response to clinical treatment. CONCLUSIONThis is the first report of inflammatory pseudotumor of the carotid artery in a patient diagnosed with TAK. Adequate pharmacological treatment and adherence to therapy may ensure better outcomes and prevent complications.