title: Takayasu arteritis in childhood: retrospective experience from a tertiary referral centre in the United Kingdom.
abstract: BACKGROUND
  Takayasu 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
  We analysed a retrospective case series of children fulfilling the TA classification criteria of the European League against Rheumatism, the Paediatric Rheumatology European Society and the Paediatric Rheumatology International Trials Organisation. Data regarding demographics, clinical features, treatments and outcomes were recorded. Descriptive statistics are 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 Indian Takayasu Arteritis Activity Score (ITAS2010) 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%) and cardiovascular (45%), neurological (36%), pulmonary (27%), skin (9%), renal (9%) and gastrointestinal (9%) involvement. At presentation, PVAS was 5/63 (1 to 13); DEI.Tak was 7/81 (2 to 12) and ITAS2010 was 9/57 (6 to 20). 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). Mortality was 27%. Young age at disease onset (<5 years old) and permanent PVDI scores≥3 were significantly associated with mortality risk (P=0.024).
  
  
  CONCLUSIONS
  TA is a rare and potentially life-threatening large-vessel vasculitis. Improved awareness of TA is essential to secure a timely diagnosis. Although the evidence base for the treatment of TA in children is weak, we found that it is essential to treat it aggressively because our data emphasise that the mortality and morbidity in the paediatric population remains high.
authors: UCL Institute of Child Health, 30 Guilford Street and Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N1EH, UK. d.eleftheriou@ucl.ac.uk.
authors: Despina
authors: D
authors: Eleftheriou
authors: UCL Institute of Child Health, 30 Guilford Street and Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N1EH, UK. giulia.varnier@gosh.nhs.uk.|Department of Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. giulia.varnier@gosh.nhs.uk.
authors: Giulia
authors: G
authors: Varnier
authors: Department of Paediatrics and Adolescent Medicine, Paediatric Rheumatology Unit, Charles University in Prague and General University Hospital in Prague, Prague, Czech Republic. dolezalova.pavla@vfn.cz.
authors: Pavla
authors: P
authors: Dolezalova
authors: Department of Paediatric Rheumatology, Sheffield Children's Hospital, Sheffield, South Yorkshire S10 2TH, UK. ammcmahon72@googlemail.com.
authors: Anne-Marie
authors: AM
authors: McMahon
authors: Department of Paediatric Rheumatology, Sheffield Children's Hospital, Sheffield, South Yorkshire S10 2TH, UK. Muthana.Al-Obaidi@sch.nhs.uk.
authors: Muthana
authors: M
authors: Al-Obaidi
authors: UCL Institute of Child Health, 30 Guilford Street and Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N1EH, UK. p.brogan@ucl.ac.uk.
authors: Paul A
authors: PA
authors: Brogan
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003952:Diagnostic Imaging / Q000379:methods*; D057510:Endovascular Procedures / Q000379:methods*; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods*; D015994:Incidence; D007223:Infant; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013625:Takayasu Arteritis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D062606:Tertiary Care Centers; D006113:United Kingdom / Q000453:epidemiology