Ehlers-Danlos syndromes: State of the art on clinical practice guidelines

Alberto Sulli, Rosaria Talarico, Carlo Alberto Scirè, Tadej Avcin, Marco Castori, Alessandro Ferraris, Charissa Frank, Jürgen Grunert, Sabrina Paolino, Stefano Bombardieri, Matthias Schneider, Vanessa Smith, Maurizio Cutolo, Marta Mosca, Fransiska Malfait

Research output: Contribution to journalReview articlepeer-review


Objective To report the effort of the European Reference Network for Rare and Complex CONnective tissue and musculoskeletal diseases NETwork working group on Ehlers-Danlos syndromes (EDS) and related disorders to assess current available clinical practice guidelines (CPGs) specifically addressed to EDS, in order to identify potential clinician and patient unmet needs. Methods Systematic literature search in PUBMED and EMBASE based on controlled terms (MeSH and Emtree) and keywords of the disease and publication type (CPGs). All the published articles were revised in order to identify existing CPGs on diagnosis, monitoring and treatment of EDS. Results Literature revision detected the absence of papers reporting good quality CPGs to optimise EDS patient care. The current evidence-based literature regarding clinical guidelines for the EDS was limited in size and quality, and there is insufficient research exploring the clinical features and interventions, and clinical decision-making are currently based on theoretical and limited research evidences. Conclusions Many clinician and patient unmet needs have been identified.

Original languageEnglish
Article numbere000790
JournalRMD Open
Publication statusPublished - Oct 1 2018


  • clinical practice guidelines
  • Ehlers-Danlos syndromes
  • European Reference Networks
  • unmet needs

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology


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