[Takayasu arteritis: a chronic vasculitis that is rare in children]

Abstract

Takayasu arteritis was diagnosed in two children, a 15-year-old girl and a 10-year-old boy. The girl had suffered from fatigue, malaise, abdominal pain and weight loss for several months, but no clear cause could be found. A few weeks later, when a blood pressure of 222/155 mmHg was measured, the possibility of renal artery stenosis was considered and imaging studies revealed indications for Takayasu's disease. The patient was given methylprednisolone followed by a combination of prednisone and, initially, cyclophosphamide, later methotrexate. This resulted in a clinical remission of the inflammatory process. The boy presented with increasing fatigue and variable episodes of fever. After 3 years, sarcoidosis or Castleman's disease were considered. Imaging studies revealed aortic stenosis. He underwent stenting of the involved vessel segment. Takayasu arteritis is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. Especially in the early phase, the symptoms often are non-specific. One should look for hypertension, blood pressure differences between the two arms, decreased peripheral pulsation or bruits over the aorta and its major branches. Radiological examination may consist ofangiography, magnetic resonance imaging or CT-scans. Treatment consists of corticosteroids and other immunosuppressants, such as cyclophosphamide, methotrexate, azathioprine, and antagonists of tumour-necrosis factor alpha. In addition, balloon dilatation or stenting is often necessary.