Title :

Systemic sclerosis: state of the art on clinical practice guidelines.

Author(s) :

Smith, Vanessa [Auteur]
Scire Carlo, Alberto [Auteur]
Talarico, Rosaria [Auteur]
Airo, Paolo [Auteur]
Alexander, Tobias [Auteur]
Allanore, Yannick [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Bruni, Cosimo [Auteur]
Codullo, Veronica [Auteur]
Dalm, Virgil [Auteur]
De Vries-Bouwstra, Jeska [Auteur]
Della Rossa, Alessandra [Auteur]
Distler, Oliver [Auteur]
Galetti, Ilaria [Auteur]
Launay, David [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Lepri, Gemma [Auteur]
Mathian, Alexis [Auteur]
Mouthon, Luc [Auteur]
Ruaro, Barbara [Auteur]
Sulli, Alberto [Auteur]
Tincani, Angela [Auteur]
Vandecasteele, Els [Auteur]
Vanhaecke, Amber [Auteur]
Vanthuyne, Marie [Auteur]
Van Den Hoogen, Frank [Auteur]
Van Vollenhoven, Ronald [Auteur]
Voskuyl Alexandre, E [Auteur]
Zanatta, Elisabetta [Auteur]
Bombardieri, Stefano [Auteur]
Burmester, Gerd [Auteur]
Fonseca Joao, Eurico [Auteur]
Frank, Charissa [Auteur]
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Houssiau, Frederic [Auteur]
Muller-Ladner, Ulf [Auteur]
Schneider, Matthias [Auteur]
Van Laar Jacob, M [Auteur]
Vieira, Ana [Auteur]
Cutolo, Maurizio [Auteur]
Mosca, Marta [Auteur]
Matucci-Cerinic, Marco [Auteur]

Journal title :

RMD Open : Rheumatic & Musculoskeletal Diseases

Abbreviated title :

RMD Open

Volume number :

4

Pages :

e000782

Publication date :

2018-01-01

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Inserm
Université de Lille
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Research team(s) :

Immunity, inflammation and fibrsis in auto and allo-reactivity

Submission date :

2019-03-01T14:07:52Z
2023-11-27T13:23:49Z