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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 and Czirjak, Laszlo, et al. (2017) In Annals of the Rheumatic Diseases 76. p.1327-1339
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... (More)

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.

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Annals of the Rheumatic Diseases
volume
76
pages
1327 - 1339
publisher
British Medical Association
external identifiers
  • scopus:84995487524
  • wos:000405191600008
ISSN
0003-4967
DOI
10.1136/annrheumdis-2016-209909
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English
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yes
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05ffc73d-5a3d-4169-8510-6237a8ad36b9
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2016-12-05 11:23:38
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2018-10-14 04:34:53
@article{05ffc73d-5a3d-4169-8510-6237a8ad36b9,
  abstract     = {<p>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.</p>},
  author       = {Kowal-Bielecka, Otylia and Fransen, Jaap and Avouac, Jerome and Becker, Mike and Kulak, Agnieszka and Allanore, Yannick and Distler, Oliver and Clements, Philip and Cutolo, Maurizio and Czirjak, Laszlo and Damjanov, Nemanja and del Galdo, Francesco and Denton, Christopher P. and Distler, Jörg H W and Foeldvari, Ivan and Figelstone, Kim and Frerix, Marc and Furst, Daniel E. and Guiducci, Serena and Hunzelmann, Nicolas and Khanna, Dinesh and Matucci-Cerinic, Marco and Herrick, Ariane L. and van den Hoogen, Frank and van Laar, Jacob M. and Riemekasten, Gabriela and Silver, Richard and Smith, Vanessa and Sulli, Alberto and Tarner, Ingo and Tyndall, Alan and Welling, Joep and Wigley, Frederic and Valentini, Gabriele and Walker, Ulrich A. and Zulian, Francesco and Müller-Ladner, Ulf and Daikeler, Thomas and Lanciano, Elisabetta and Becvár, Radim and Tomcik, Michal and Gindzienska-Sieskiewicz, Ewa and Iudici, Michele and Rednic, Simona and Vlachoyiannopoulos, Panayiotis G. and Caporali, Roberto and Carreira, Patricia E. and Novak, Srdan and Minier, Tünde and Kucharz, Eugene J. and Gabrielli, Armando and Moroncini, Gianluca and Airo, Paolo and Hesselstrand, Roger and Martinovic, Duska and Radic, Mislav and Marasovic-Krstulovic, Daniela and Braun-Moscovici, Yolanda and Monaco, Andrea Lo and Morovic-Vergles, Jadranka and Culo, Melanie I. and Henes, Jörg and Santamaria, Vera Ortiz and Heitmann, Stefan and Krasowska, Dorota and Michalska-Jakubus, Malgorzata and Seidel, Matthias F. and Klinik, Medizinische and Hasler, Paul and Da Silva, José A Pereira and Salvador, Maria J. and Stamenkovic, Bojana and Stankovic, Aleksandra and Tikly, Mohammed and Ananieva, Lidia P. and Beretta, Lorenzo and Szucs, Gabriella and Szamosi, Szilvia and de la Puente Bujidos, Carlos and Midtvedt, Øyvind and Hoffmann-Vold, Anna Maria and Launay, David and Hachulla, Eric and Riccieri, Valeria and Ionescu, Ruxandra and Opris, Daniela and Mihai, Carina and Herrgott, Ilka and Beyer, Christian and Ingegnoli, Francesca and von Mühlen, Carlos Alberto and Alegre-Sancho, Juan José and Beltran-Catalan, Emma and Aringer, Martin and Fantana, Julia and Leuchten, Nicolai and Tausche, Anne Kathrin and Langhe, Ellen De and Vanthuyne, Marie and Anic, Branimir and Barešic, Marko and Mayer, Miroslav and üprus, Maria and Otsa, Kati and Yavuz, Sule and Granel, Brigitte and Jimenez, Sergio A. and Popa, Serghei and Agachi, Svetlana and Zenone, Thierry and Stebbings, Simon and Dockerty, Joanne and Vacca, Alessandra and Schollum, Joanna and Veale, Douglas J. and Toloza, Sergio and Xu, Dong and Olas, Jacek and Rosato, Edoardo and Foti, Rosario and Adler, Sabine and Dan, Diana and Wiesik-Szewczyk, Ewa and Olesinska, Marzena and Kayser, Cristiane and Fathi, Nihal and de la Peña Lefebvre, Paloma García and Imbert, Bernard},
  issn         = {0003-4967},
  language     = {eng},
  pages        = {1327--1339},
  publisher    = {British Medical Association},
  series       = {Annals of the Rheumatic Diseases},
  title        = {Update of EULAR recommendations for the treatment of systemic sclerosis},
  url          = {http://dx.doi.org/10.1136/annrheumdis-2016-209909},
  volume       = {76},
  year         = {2017},
}