P088 Prolonged fever in a boy revealing Takayasu disease: a case report

Background Takayasu's arteritis (TA) is a chronic inflammatory vasculitis of unknown origin. It affects the large vessels, especially the aorta, its main branches, and the pulmonary arteries. It begins acutely in children with severe general manifestations. The diagnosis of TA remains a challenge to clinicians due to many reasons such as its rarity, its great clinical polymorphism, and the lack of specific biological criteria. Case report A 13-years old boy with no pathological history, was admitted for prolonged fever with intermittent abdominal pain. The clinical examination has found a conscious boy, febrile with a BP of 130/90mmhg, a normal heart rate, and stable respiratory function. The cardiovascular examination noted a decrease in pedal pulses, without signs of ischaemia or necrosis, and the cardiac auscultation was normal. Examination of the lymph node areas has shown upper and left later cervical adenopathy measuring 1.7 cm in long axis, mobile, and painless. All joints were free. The complementary biological workups revealed an inflammatory syndrome (ESR: 120 mm, fibrinogen: 5 g/l, microcytic hypochromic anaemia at 9 g/dl, ferritin: 1051, low serum iron: 11µg/dl and thrombocytosis: 692 000 elements/l). Chest X-ray showed a dilated aortic button. A thoracic angioscan revealed a peri-aortitis more evident at the level of the emergence of the mesenteric artery with a pseudo-aneurysmal aspect and arteritis of the right primary carotid artery. Echocardiography was performed and showed a dilated aspect of the aorta with irregular wall. Renal ultrasound was normal. The diagnosis of Takayasu disease was made on the basis of clinical biological and essentially radiological arguments according to the diagnostic criteria of the American College of Rheumatology. The patient was treated with corticosteroid (prednisone: 2 mg/kg/d) for one month then then gradually reduced doses The disease course was marked by several relapses and the patient was then treated with mycophenolate mofetil (cellcept). Conclusion Takaysu's disease is a chronic inflammatory vasculitis of unknown origin, which affects the large- vessels, mainly the aorta, its main branches, and the pulmonary arteries; but it remains rare in children and its treatment is not well codified.