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Takayasu arteritis: key clinical factors for early diagnosis
  1. J Forsey1,
  2. G Dhandayuthapani1,
  3. MCK Hamilton2,
  4. RP Martin1,
  5. EJ Tizard3,
  6. AV Ramanan4
  1. 1Bristol Congenital Heart Centre, Bristol Royal Hospital for Children, Bristol, UK
  2. 2Department of Clinical Radiology, Bristol Royal Infirmary, Bristol, UK
  3. 3Department of Paediatric Nephrology, Bristol Royal Hospital for Children, Bristol, UK
  4. 4Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK
  1. Correspondence to Dr AV Ramanan, Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol BS2 8BJ, UK; avramanan{at}hotmail.com

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Introduction

Takayasu arteritis is a large and medium size vessel vasculitis involving the aorta, its major branches and the pulmonary arteries. It is the most common large vessel vasculitis in childhood and is of unknown aetiology. The presentation is often non-specific, making a timely diagnosis difficult. It predominantly presents in the second or third decade of life, although it has been described in children1 and there is a strong female predisposition (8:1).2

The clinical presentation is varied but can be divided into early (prepulseless) and late (pulseless) phases. The challenge for the clinician is to consider Takayasu arteritis during the early phase when the symptoms are non-specific and often systemic.

We highlight three cases, seen at a tertiary referral centre for paediatric rheumatology and nephrology, to illustrate the difficulty posed by the presentation of Takayasu arteritis and the important features that aid early diagnosis. Initial clinical findings are summarised in table 1.

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Table 1

Clinical findings and investigations at presentation

Case 1

A 15-year-old Caucasian girl presented with a 2-month history of malaise and lethargy and a 1-month history of non-specific central chest pain associated with shortness of breath. Her examination revealed pallor and a grade 2/6 systolic murmur at the mid left sternal edge. Baseline observations were normal including blood pressure. There was no relevant family history. Her initial presentation was thought to be secondary to an underlying autoimmune condition and her laboratory investigations revealed a C reactive protein (CRP) and erythrocyte sedimentation rate (ESR) of 323 mg/l and 138 mm/h, respectively. There was a normocytic normochromic anaemia (Hb 9.6 g/dl) and a weakly positive antinuclear antibody (ANA) (1 in 80).

Initial treatment with oral prednisone (1 mg/kg) improved her symptoms, but any attempts to wean her off steroids resulted in a relapse. In light of the difficulty in reducing steroids, …

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