TY - JOUR
T1 - Systemic sclerosis: State of the art on clinical practice guidelines
AU - Smith, Vanessa
AU - Scirè, Carlo Alberto
AU - Talarico, Rosaria
AU - Airo, Paolo
AU - Alexander, Tobias
AU - Allanore, Yannick
AU - Bruni, Cosimo
AU - Codullo, Veronica
AU - Dalm, Virgil
AU - de Vries-Bouwstra, Jeska
AU - Della Rossa, Alessandra
AU - Distler, Oliver
AU - Galetti, Ilaria
AU - Launay, David
AU - Lepri, Gemma
AU - Mathian, Alexis
AU - Mouthon, Luc
AU - Ruaro, Barbara
AU - Sulli, Alberto
AU - Tincani, Angela
AU - Vandecasteele, Els
AU - Vanhaecke, Amber
AU - Vanthuyne, Marie
AU - van den Hoogen, Frank
AU - van Vollenhoven, Ronald
AU - Voskuyl, Alexandre E.
AU - Zanatta, Elisabetta
AU - Bombardieri, Stefano
AU - Burmester, Gerd
AU - Eurico, Fonseca João
AU - Frank, Charissa
AU - Hachulla, Eric
AU - Houssiau, Frederic
AU - Mueller-Ladner, Ulf
AU - Schneider, Matthias
AU - van Laar, Jacob M.
AU - Vieira, Ana
AU - Cutolo, Maurizio
AU - Mosca, Marta
AU - Matucci-Cerinic, Marco
PY - 2018
Y1 - 2018
N2 - 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 nonpharmacological) 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.
AB - 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 nonpharmacological) 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.
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85055708612&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/30402270
U2 - https://doi.org/10.1136/rmdopen-2018-000782
DO - https://doi.org/10.1136/rmdopen-2018-000782
M3 - Review article
C2 - 30402270
SN - 2056-5933
VL - 4
JO - RMD OPEN
JF - RMD OPEN
M1 - e000782
ER -