title: Raynaud's syndrome in children: systematic review and development of recommendations for assessment and monitoring.
abstract: OBJECTIVE
  To develop recommendations for investigation and monitoring of children with Raynaud's syndrome, based on paediatric evidence collated by a systematic review.
  
  
  METHODS
  A systematic review was undertaken to establish the paediatric evidence for assessment and monitoring of Raynaud's syndrome. An expert panel including members of the Paediatric Rheumatology European Society (PRES) Scleroderma Working Group, were invited to a consensus meeting where recommendations were developed based on evidence graded by the systematic review and where evidence was lacking, consensus opinion. A nominal technique was used where 75% consensus was taken as agreement.
  
  
  RESULTS
  The expert panel recommended testing anti-nuclear antibody (ANA), more specific antibodies associated with connective tissue disease and nail-fold capillaroscopy in all children presenting with Raynaud's syndrome as data suggests these can be risk factors for evolution into a connective tissue disease. The frequency of follow-up recommended depends on presence of these risk factors with the aim to detect evolving connective tissue disease early in high risk individuals. Those with no abnormalities on capillaroscopy and negative autoantibodies were deemed low risk of progression, whereas those with ANA positivity, specific autoantibodies and/or nailfold capillary changes were deemed high risk and more frequent follow-up was recommended.
  
  
  CONCLUSIONS
  Recommendations, primarily based on consensus opinion, were agreed regarding investigation and monitoring of children who present with Raynaud's syndrome. Further prospective studies are needed to better define the risk factors for progression to connective tissue disease.
authors: Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
authors: Clare E
authors: CE
authors: Pain
authors: Semmelweis University, Budapest, Hungary.
authors: Tamás
authors: T
authors: Constantin
authors: University Children's Hospital Ljubljana, Slovenia.
authors: Natasa
authors: N
authors: Toplak
authors: University Children's Hospital, Tuebingen, Germany.
authors: Monica
authors: M
authors: Moll
authors: Universitätsklinikum Hamburg-Eppendorf, Germany.
authors: Christof
authors: C
authors: Iking-Konert
authors: Universidade Federal de São Paulo, Brazil.
authors: Daniella P
authors: DP
authors: Piotto
authors: Istanbul Kanuni Sultan Süleyman Education and Research Hospital, Turkey.
authors: Nuray
authors: N
authors: Aktay Ayaz
authors: Charles University, Prague, Czech Republic.
authors: Dana
authors: D
authors: Nemcova
authors: Department of Paediatric Dermatology, Cath. Children's Hospital, Wilhelmstift, Hamburg, Germany.
authors: Peter H
authors: PH
authors: Hoeger
authors: University of Genoa, Italy.
authors: Maurizio
authors: M
authors: Cutolo
authors: Ghent University Hospital, Belgium.
authors: Vanessa
authors: V
authors: Smith
authors: Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany. foeldvari@t-online.de.
authors: Ivan
authors: I
authors: Foeldvari
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000974:Antibodies, Antinuclear / Q000097:blood; D015415:Biomarkers / Q000097:blood; D002648:Child; D032921:Consensus; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D020678:Microscopic Angioscopy / Q000592:standards*; D010372:Pediatrics / Q000592:standards*; D011237:Predictive Value of Tests; D011379:Prognosis; D011928:Raynaud Disease / Q000097:blood / Q000175:diagnosis* / Q000628:therapy; D012219:Rheumatology / Q000592:standards*; D012307:Risk Factors; D012698:Serologic Tests / Q000592:standards*
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers