Macaroni sign and carotid occlusion in Takayasu ’s arteritis

A 29-year-old female from Guinea-Bissau was referred for Rheumatology observation with a 3-year history of fatigue, myalgia, dizziness, headache and anterior chest pain. On physical examination, the right carotid artery (CA) pulse was extremely diminished and left CA pulse absent. Laboratory tests revealed a normocytic anaemia (Hb 10.5  g/dl) and elevated erythrocyte sedimentation rate (93 mm/h) and C-reactive protein (0.55 mg/dl). Ultrasound of the right common CA showed a homogeneous and mid-echogenic circumferential wall thickening compatible with the ‘macaroni sign’ (Fig. 1A). Ultrasound of the left common CA presented a complete arterial occlusion (Fig. 1B). Takayasu ’s arteritis (TAK) is a rare large-vessel vasculitis occurring mainly in young women and commonly affecting the supra-aortic arteries. Presence of the ‘macaroni sign’ on CA ultrasound is highly suggestive of this diagnosis [1]. This case highlights the utility of ultrasound in diagnosing patients with TAK, as well as it emphasizes the need for an early referral to avoid progression to severe vascular lesions [2].
Source: Rheumatology - Category: Rheumatology Source Type: research