The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.
Update of EULAR recommendations for the treatment of systemic sclerosis / Kowal Bielecka, Otylia; Fransen, Jaap; Avouac, Jerome; Becker, Mike; Kulak, Agnieszka; Allanore, Yannick; Distler, Oliver; Clements, Philip; Cutolo, Maurizio; Czirjak, Laszlo; Damjanov, Nemanja; del Galdo, Francesco; Denton, Christopher P.; Distler, Jörg H. W.; Foeldvari, Ivan; Figelstone, Kim; Frerix, Marc; Furst, Daniel E.; Guiducci, Serena; Hunzelmann, Nicolas; Khanna, Dinesh; Matucci Cerinic, Marco; Herrick, Ariane L.; van den Hoogen, Frank; van Laar, Jacob M.; Riemekasten, Gabriela; Silver, Richard; Smith, Vanessa; Sulli, Alberto; Tarner, Ingo; Tyndall, Alan; Welling, Joep; Wigley, Frederic; Valentini, Gabriele; Walker, Ulrich A.; Zulian, Francesco; Müller Ladner, Ulf; Daikeler, Thomas; Lanciano, Elisabetta; Becvár, Radim; Tomcik, Michal; Gindzienska Sieskiewicz, Ewa; Iudici, Michele; Rednic, Simona; Vlachoyiannopoulos, Panayiotis G.; Caporali, Roberto; Carreira, Patricia E.; Novak, Srdan; Minier, Tünde; Kucharz, Eugene J.; Gabrielli, Armando; Moroncini, Gianluca; Airo, Paolo; Hesselstrand, Roger; Martinovic, Duska; Radic, Mislav; Marasovic Krstulovic, Daniela; Braun Moscovici, Yolanda; Monaco, Andrea Lo; Morovic Vergles, Jadranka; Culo, Melanie I.; Henes, Jörg; Santamaria, Vera Ortiz; Heitmann, Stefan; Krasowska, Dorota; Michalska Jakubus, Malgorzata; Seidel, Matthias F.; Klinik III, Medizinische; Hasler, Paul; Da Silva, José A. Pereira; Salvador, Maria J.; Stamenkovic, Bojana; Stankovic, Aleksandra; Tikly, Mohammed; Ananieva, Lidia P.; Beretta, Lorenzo; Szucs, Gabriella; Szamosi, Szilvia; de la Puente Bujidos, Carlos; Midtvedt, Øyvind; Hoffmann Vold, Anna Maria; Launay, David; Hachulla, Eric; Riccieri, Valeria; Ionescu, Ruxandra; Opris, Daniela; Mihai, Carina; Herrgott, Ilka; Beyer, Christian; Ingegnoli, Francesca; von Mühlen, Carlos Alberto; Alegre Sancho, Juan José; Beltran Catalan, Emma; Aringer, Martin; Fantana, Julia; Leuchten, Nicolai; Tausche, Anne Kathrin; Langhe, Ellen De; Vanthuyne, Marie; Anic, Branimir; Barešic, Marko; Mayer, Miroslav; Üprus, Maria; Otsa, Kati; Yavuz, Sule; Granel, Brigitte; Jimenez, Sergio A.; Popa, Serghei; Agachi, Svetlana; Zenone, Thierry; Stebbings, Simon; Dockerty, Joanne; Vacca, Alessandra; Schollum, Joanna; Veale, Douglas J.; Toloza, Sergio; Xu, Dong; Olas, Jacek; Rosato, Edoardo; Foti, Rosario; Adler, Sabine; Dan, Diana; Wiesik Szewczyk, Ewa; Olesinska, Marzena; Kayser, Cristiane; Fathi, Nihal; de la Peña Lefebvre, Paloma García; Imbert, Bernard. - In: ANNALS OF THE RHEUMATIC DISEASES. - ISSN 0003-4967. - STAMPA. - 76:8(2017), pp. 1327-1338. [10.1136/annrheumdis-2016-209909]
Update of EULAR recommendations for the treatment of systemic sclerosis
RICCIERI, Valeria;ROSATO, Edoardo;
2017
Abstract
The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.| File | Dimensione | Formato | |
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