RT Journal Article
SR Electronic
T1 Systemic sclerosis: state of the art on clinical practice guidelines
JF RMD Open
JO RMD Open
FD EULAR
SP e000782
DO 10.1136/rmdopen-2018-000782
VO 4
IS Suppl 1
A1 Vanessa Smith
A1 Carlo Alberto Scirè
A1 Rosaria Talarico
A1 Paolo Airo
A1 Tobias Alexander
A1 Yannick Allanore
A1 Cosimo Bruni
A1 Veronica Codullo
A1 Virgil Dalm
A1 Jeska De Vries-Bouwstra
A1 Alessandra Della Rossa
A1 Oliver Distler
A1 Ilaria Galetti
A1 David Launay
A1 Gemma Lepri
A1 Alexis Mathian
A1 Luc Mouthon
A1 Barbara Ruaro
A1 Alberto Sulli
A1 Angela Tincani
A1 Els Vandecasteele
A1 Amber Vanhaecke
A1 Marie Vanthuyne
A1 Frank Van den Hoogen
A1 Ronald Van Vollenhoven
A1 Alexandre E Voskuyl
A1 Elisabetta Zanatta
A1 Stefano Bombardieri
A1 Gerd Burmester
A1 João Eurico Fonseca
A1 Charissa Frank
A1 Eric Hachulla
A1 Frederic Houssiau
A1 Ulf Mueller-Ladner
A1 Matthias Schneider
A1 Jacob M van Laar
A1 Ana Vieira
A1 Maurizio Cutolo
A1 Marta Mosca
A1 Marco Matucci-Cerinic
YR 2019
UL http://rmdopen.bmj.com/content/4/Suppl_1/e000782.abstract
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 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.