Abstract

Takayasu arteritis is a potentially life threatening vasculitis that characterized by granulomatous inflammation of large vessels including aorta, its major branches and pulmonary arteries. True incidence of TA is not known in children, since TA is very rare; however, incidence of TA has been estimated as 0.8-2.6 per million children. Making a diagnosis is more difficult in childhood compared with adults due to non-specific clinical findings. The diagnosis of TA is done based on clinical criteria, laboratory investigations support and radiologic images confirm the diagnosis. Corticosteroids with other immunosuppressive drugs are used for treatment and biological agents are given to resistant cases. In this report, it was aimed to draw attention to a potentially fatal disease that frequently diagnostic delays and therapeutic difficulties occur. We, herein reported signs, symptoms, imaging methods and treatment modalities of two children, a 12 year-old male and a 13 year-old female patients, that presented with non-specific initial symptoms and diagnosed as TA with clinical findings and radiological images

Highlights

  • Takayasu arteritis (TA) known as “pulseless disease” is a chronic, idiopathic granulomatous vasculitis that involves large vessels

  • 19 months of delay occur in the diagnosis of TA cases

  • The diagnosis of TA is usually difficult for physicians and an average of 19 months of diagnostic delay has been reported [1]

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Summary

Introduction

Takayasu arteritis (TA) known as “pulseless disease” is a chronic, idiopathic granulomatous vasculitis that involves large vessels. The average presentation age is 11.4 years and 20% of cases are diagnosed before the age of 19 years. 19 months of delay occur in the diagnosis of TA cases. Diagnosis and effective treatment can prevent compliactions related to the disease [1]. Received: 01.08.2013, Accepted: 04.08.2013 threatening vasculitis with the approximately mortality rate of 35% in children [2]. The disease has an acute early phase with non-specific symptoms, such as hypertension, headache, fever, muscle pain, arthralgia, night sweats and weight loss. One-third of children present with inactive disease in which there are vascular sequels instead of active vasculitis.

Takayasu arteritis in childhood
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