Journal article
Authors list: Smith, Vanessa; Scire, Carlo Alberto; Talarico, Rosaria; Airo, Paolo; Alexander, Tobias; Allanore, Yannick; Bruni, Cosimo; Codullo, Veronica; Dalm, Virgil; De Vries-Bouwstra, Jeska; Della Rossa, Alessandra; Distler, Oliver; Galetti, Ilaria; Launay, David; Lepri, Gemma; Mathian, Alexis; Mouthon, Luc; Ruaro, Barbara; Sulli, Alberto; Tincani, Angela; Vandecasteele, Els; Vanhaecke, Amber; Vanthuyne, Marie; Van den Hoogen, Frank; Van Vollenhoven, Ronald; Voskuyl, Alexandre E.; Zanatta, Elisabetta; Bombardieri, Stefano; Burmester, Gerd; Eurico, Fonseca Joao; Frank, Charissa; Hachulla, Eric; Houssiau, Frederic; Mueller-Ladner, Ulf; Schneider, Matthias; van Laar, Jacob M.; Vieira, Ana; Cutolo, Maurizio; Mosca, Marta; Matucci-Cerinic, Marco
Publication year: 2018
Journal: RMD Open: Rheumatic and Musculoskeletal Diseases
Volume number: 4
ISSN: 2056-5933
DOI Link: https://doi.org/10.1136/rmdopen-2018-000782
Publisher: BMJ Publishing Group
Abstract:
Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-) pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.
Citation Styles
Harvard Citation style: Smith, V., Scire, C., Talarico, R., Airo, P., Alexander, T., Allanore, Y., et al. (2018) Systemic sclerosis: state of the art on clinical practice guidelines, RMD OPEN, 4, Article UNSP e000782. https://doi.org/10.1136/rmdopen-2018-000782
APA Citation style: Smith, V., Scire, C., Talarico, R., Airo, P., Alexander, T., Allanore, Y., Bruni, C., Codullo, V., Dalm, V., De Vries-Bouwstra, J., Della Rossa, A., Distler, O., Galetti, I., Launay, D., Lepri, G., Mathian, A., Mouthon, L., Ruaro, B., Sulli, A., ...Matucci-Cerinic, M. (2018). Systemic sclerosis: state of the art on clinical practice guidelines. RMD OPEN. 4, Article UNSP e000782. https://doi.org/10.1136/rmdopen-2018-000782
Keywords
DEVELOPING CRITERIA; EXPERT CONSENSUS; FUNCTIONAL DISABILITY; INTERSTITIAL LUNG-DISEASE; PRACTICE PATHWAY; RECOMMENDATIONS; SIMPLE CAPILLAROSCOPIC DEFINITIONS; SKIN ULCERS; STEM-CELL TRANSPLANTATION
