TY - JOUR T1 - Systemic sclerosis: state of the art on clinical practice guidelines JF - RMD Open JO - RMD Open DO - 10.1136/rmdopen-2018-000782 VL - 4 IS - Suppl 1 SP - e000782 AU - Vanessa Smith AU - Carlo Alberto Scirè AU - Rosaria Talarico AU - Paolo Airo AU - Tobias Alexander AU - Yannick Allanore AU - Cosimo Bruni AU - Veronica Codullo AU - Virgil Dalm AU - Jeska De Vries-Bouwstra AU - Alessandra Della Rossa AU - Oliver Distler AU - Ilaria Galetti AU - David Launay AU - Gemma Lepri AU - Alexis Mathian AU - Luc Mouthon AU - Barbara Ruaro AU - Alberto Sulli AU - Angela Tincani AU - Els Vandecasteele AU - Amber Vanhaecke AU - Marie Vanthuyne AU - Frank Van den Hoogen AU - Ronald Van Vollenhoven AU - Alexandre E Voskuyl AU - Elisabetta Zanatta AU - Stefano Bombardieri AU - Gerd Burmester AU - João Eurico Fonseca AU - Charissa Frank AU - Eric Hachulla AU - Frederic Houssiau AU - Ulf Mueller-Ladner AU - Matthias Schneider AU - Jacob M van Laar AU - Ana Vieira AU - Maurizio Cutolo AU - Marta Mosca AU - Marco Matucci-Cerinic Y1 - 2019/01/01 UR - http://rmdopen.bmj.com/content/4/Suppl_1/e000782.abstract 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 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. ER -