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25 february 2015 09:23:01

 
Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the UK (Arthritis Research & Therapy)
 


IntroductionTakayasu arteritis (TA) is an idiopathic large vessel vasculitis affecting the aorta and its major branches. Although the disease rarely affects children, it does occur, even in infants. The objective of this study was to evaluate the clinical features, disease activity, treatment and outcome of childhood TA in a tertiary UK centre. Methods: Retrospective case series of children fulfilling the EULAR/PRES/PRINTO classification criteria for TA. Demographics, clinical features, treatment and outcomes were recorded. Descriptive statistics were expressed as median and range. Fisher’s exact test was used for group comparisons. The Paediatric Vasculitis Activity Score (PVAS), Paediatric Vasculitis Damage Index (PVDI), Disease Extent Index-Takayasu (DEI.TAK) and the Indian Takayasu Arteritis Score 2010 (ITAS-2010) were calculated retrospectively. Results: A total of 11 children (64% female) with age at diagnosis of 11.8 (1.3 to 17) years were identified over a 23-year period. The median time to diagnosis was 17 (0 to 132) months. The most common clinical features at presentation were: arterial hypertension (72.7%); systemic features (36%); cardiovascular (45%); neurological (36%); pulmonary (27%); skin (9%); renal (9%) and gastrointestinal involvement (9%). PVAS at presentation was 5/63 (1 to 13/63); DEITAK 7/81 (2 to 12/81) and ITAS-2010 9/57 (6 to 20/57). Treatment included corticosteroids (81.8%), combined with methotrexate in most cases (72.7%). Cyclophosphamide (36.4%) and biologic agents (45.5%) were reserved for severe and/or refractory cases. PVDI at latest follow up was 5.5/72 (3 to 15/72). Mortality was 27%; young age at disease onset (


 
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