Format

Send to

Choose Destination
RMD Open. 2018 Oct 18;4(Suppl 1):e000790. doi: 10.1136/rmdopen-2018-000790. eCollection 2018.

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

Author information

1
Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genova, Italy.
2
Rheumatology Unit, AOU Pisana, Pisa, Italy.
3
Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
4
Department of Allergology, Rheumatology and Clinical Immunology, Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
5
Division of Medical Genetics, IRCCS Casa Sollievo della Sofferenza, Foggia, Italy.
6
Medical Genetics Laboratory, Molecular Medicine Department, San Camillo Forlanini Hospital, Sapienza University, Rome, Italy.
7
Flemish Association for Hereditary Connective Tissue Disorders, Brussels, Belgium.
8
Deutsche Ehlers-Danlos Initiative e.V., Furth, Germany.
9
University of Pisa, Pisa, Italy.
10
Institute for Rheumatology, Hiller Research Unit for Rheumatology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany.
11
Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
12
Department of Internal Medicine, Ghent University, Ghent, Belgium.
13
Department of Rheumatology Unit, University of Pisa, Pisa, Italy.
14
Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.

Abstract

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.

KEYWORDS:

ERN ReCONNET; Ehlers-Danlos syndromes; European Reference Networks; clinical practice guidelines; unmet needs

Supplemental Content

Full text links

Icon for BMJ Publishing Group Icon for PubMed Central
Loading ...
Support Center