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Systemic sclerosis : state of the art on clinical practice guidelines

(2018) RMD OPEN. 4(suppl. 1).
Author
Organization
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.
Keywords
SIMPLE CAPILLAROSCOPIC DEFINITIONS, STEM-CELL TRANSPLANTATION, INTERSTITIAL LUNG-DISEASE, PRACTICE PATHWAY, FUNCTIONAL DISABILITY, DEVELOPING CRITERIA, EXPERT CONSENSUS, SKIN ULCERS, POINTS, RECOMMENDATIONS

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Citation

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Chicago
Smith, Vanessa, Carlo Alberto Scirè, Rosaria Talarico, Paolo Airo, Tobias Alexander, Yannick Allanore, Cosimo Bruni, et al. 2018. “Systemic Sclerosis : State of the Art on Clinical Practice Guidelines.” Rmd Open 4 (suppl. 1).
APA
Smith, V., Scirè, C. A., Talarico, R., Airo, P., Alexander, T., Allanore, Y., Bruni, C., et al. (2018). Systemic sclerosis : state of the art on clinical practice guidelines. RMD OPEN, 4(suppl. 1).
Vancouver
1.
Smith V, Scirè CA, Talarico R, Airo P, Alexander T, Allanore Y, et al. Systemic sclerosis : state of the art on clinical practice guidelines. RMD OPEN. 2018;4(suppl. 1).
MLA
Smith, Vanessa et al. “Systemic Sclerosis : State of the Art on Clinical Practice Guidelines.” RMD OPEN 4.suppl. 1 (2018): n. pag. Print.
@article{8600434,
  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.},
  articleno    = {e000782},
  author       = {Smith, Vanessa and Scirè, Carlo Alberto and Talarico, Rosaria and Airo, Paolo and Alexander, Tobias and Allanore, Yannick and Bruni, Cosimo and Codullo, Veronica and Dalm, Virgil and De Vries-Bouwstra, Jeska and Della Rossa, Alessandra and Distler, Oliver and Galetti, Ilaria and Launay, David and Lepri, Gemma and Mathian, Alexis and Mouthon, Luc and Ruaro, Barbara and Sulli, Alberto and Tincani, Angela and Vandecasteele, Els and Vanhaecke, Amber and Vanthuyne, Marie and Van den Hoogen, Frank and Van Vollenhoven, Ronald and Voskuyl, Alexandre E and Zanatta, Elisabetta and Bombardieri, Stefano and Burmester, Gerd and Eurico, Fonseca João and Frank, Charissa and Hachulla, Eric and Houssiau, Frederic and Mueller-Ladner, Ulf and Schneider, Matthias and van Laar, Jacob M and Vieira, Ana and Cutolo, Maurizio and Mosca, Marta and Matucci-Cerinic, Marco},
  issn         = {2056-5933},
  journal      = {RMD OPEN},
  keywords     = {SIMPLE CAPILLAROSCOPIC DEFINITIONS,STEM-CELL TRANSPLANTATION,INTERSTITIAL LUNG-DISEASE,PRACTICE PATHWAY,FUNCTIONAL DISABILITY,DEVELOPING CRITERIA,EXPERT CONSENSUS,SKIN ULCERS,POINTS,RECOMMENDATIONS},
  language     = {eng},
  number       = {suppl. 1},
  pages        = {9},
  title        = {Systemic sclerosis : state of the art on clinical practice guidelines},
  url          = {http://dx.doi.org/10.1136/rmdopen-2018-000782},
  volume       = {4},
  year         = {2018},
}

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