Macaroni sign and takayasu arteritis in a patient with tubercolosis: an easy tool for diagnosis

Abstract Takayasu arteritis (TKA) is a chronic systemic autoimmune disease characterised by inflammation of the aorta and major branch vessels. Carotid Artery (CA) lesions in TKA, known as the ‘macaroni sign’, are characterised by global thickening of the common CA wall and lumen narrowing. There has been speculation that there is a link between TKA and tuberculosis (TB). Both illnesses cause arterial wall changes. We describe a TKA case diagnosed based on the presence of the ‘macaroni sign’ on CU in a young Asian women 30 years old with a diagnosis of latent TB. She has not previous histiry of disease and visited the hospital with complaints dyspnoea and carotidynia. At admission, body temperature, blood pressure and heart rate were 36.8°C, 112/60mm Hg on her right arm (left, 84/60 mmHg) and 76 beats/min, respectively. The pulse in her left radial artery was not palpable. Physical examination revealed spontaneous pain and tenderness on both sides of the patient’s neck. Blood test results demonstrated an elevated inflammatory response C–reactive protein, 8.0 mg/dL (normal range: less than 0.5 mg/L); erythrocyte sedimentation rate (ESR), 120 mm/hour. ANA, ENA and ASMA profile were negative. Quantiferon TB gold was positive. We suspected TKA and performed a CU, which revealed marked thickening of the intima/media of the bilateral common CAs and a positive macaroni sign (figure 1, Panel A and B). After a detailed evaluation of the systemic vessels the patient was diagnosed with TKA Type I limited to Branches from the aortic arch. The FDG–PET/CT demonstrated an increase in the glucose uptake in thoracic aorta, subclavian and right CA (SUV 4.5). The patient was treated with oral steroids at a dose of 0.8mg/kg and Isoniazid. After 6 months she showed a disappearance of carotidynia and dyspnoea, with a reduction in C–reactive protein, 1.5 mg/ dL and ESR=39 mm/hour. The FDG–PET/CT demonstrated a reduction in the glucose uptake in thoracic aorta, subclavian and right CA (SUV 3.5). Although MRI is usually recommended for the definitive diagnosis, the ‘macaroni sign’ identified by CU is a low–cost, simple and non–invasive method that can be easily performed during initial hospital visits. In addition, a clear correlation between MRI and CT angiography findings and disease activity or progression has not been found for TKA yet. Therefore, methods using ultrasound in combination with ESR should be considered easy and useful for diagnosis and clinical follow–up.