Update of EULAR recommendations for the treatment of systemic sclerosis
Otylia Kowal-Bielecka, Jaap Fransen, Jerome Avouac, Mike Becker, Agnieszka Kulak, Yannick Allanore, Oliver Distler, Philip Clements, Maurizio Cutolo, Laszlo Czirjak, Nemanja Damjanov, Francesco Del Galdo, Christopher P Denton, Jörg H W Distler, Ivan Foeldvari, Kim Figelstone, Marc Frerix, Daniel E Furst, Serena Guiducci, Nicolas Hunzelmann, Dinesh Khanna, Marco Matucci-Cerinic, Ariane L Herrick, Frank van den Hoogen, Jacob M van Laar, Gabriela Riemekasten, Richard Silver, Vanessa Smith, Alberto Sulli, Ingo Tarner, Alan Tyndall, Joep Welling, Frederic Wigley, Gabriele Valentini, Ulrich A Walker, Francesco Zulian, Ulf Müller-Ladner, EUSTAR Coauthors, Thomas Daikeler, Elisabetta Lanciano, Radim Bečvář, Michal Tomcik, Ewa Gińdzieńska-Sieśkiewicz, Giovanna Cuomo, Michele Iudici, Simona Rednic, Panayiotis G Vlachoyiannopoulos, Roberto Caporali, Patricia E Carreira, Servicio de Reumatología, Srdan Novak, Tünde Minier, Eugene J Kucharz, Armando Gabrielli, Gianluca Moroncini, Paolo Airo', Roger Hesselstrand, Duska Martinovic, Mislav Radić, Daniela Marasovic-Krstulovic, Yolanda Braun-Moscovici, Alexandra Balbir-Gurman, Andrea Lo Monaco, Paola Caramaschi, Jadranka Morović-Vergles, Melanie I Čulo, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Małgorzata Michalska-Jakubus, Matthias F Seidel, Paul Hasler, Klinik Für Rheumatologie, José A Pereira Da Silva, Maria J Salvador, Bojana Stamenkovic, Aleksandra Stankovic, Mohammed Tikly, Lidia P Ananieva, Lorenzo Beretta, Gabriella Szucs, Szilvia Szamosi, Carlos de la Puente Bujidos, Servicio de Reumatología, Øyvind Midtvedt, Anna-Maria Hoffmann-Vold, David Launay, Eric Hachulla, Valeria Riccieri, Ruxandra Ionescu, Daniela Opris, Carina Mihai, Ilka Herrgott, Christian Beyer, Francesca Ingegnoli, Carlos Alberto von Mühlen, Juan José Alegre-Sancho, Emma Beltrán-Catalán, Martin Aringer, Julia Fantana, Nicolai Leuchten, Anne-Kathrin Tausche, Ellen De Langhe, Marie Vanthuyne, Branimir Anic, Marko Barešić, Miroslav Mayer, Maria Üprus, Kati Otsa, Sule Yavuz, Brigitte Granel, Valderilio F Azevedo, Carolina Muller, Sergio A Jimenez, Serghei Popa, Svetlana Agachi, Thierry Zenone, Simon Stebbings, Joanne Dockerty, Alessandra Vacca, Joanna Schollum, Douglas J Veale, Sergio Toloza, Dong Xu, Jacek Olas, Edoardo Rosato, Rosario Foti, Sabine Adler, Diana Dan, Ewa Wiesik-Szewczyk, Marzena Olesińska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Bernard Imbert, Otylia Kowal-Bielecka, Jaap Fransen, Jerome Avouac, Mike Becker, Agnieszka Kulak, Yannick Allanore, Oliver Distler, Philip Clements, Maurizio Cutolo, Laszlo Czirjak, Nemanja Damjanov, Francesco Del Galdo, Christopher P Denton, Jörg H W Distler, Ivan Foeldvari, Kim Figelstone, Marc Frerix, Daniel E Furst, Serena Guiducci, Nicolas Hunzelmann, Dinesh Khanna, Marco Matucci-Cerinic, Ariane L Herrick, Frank van den Hoogen, Jacob M van Laar, Gabriela Riemekasten, Richard Silver, Vanessa Smith, Alberto Sulli, Ingo Tarner, Alan Tyndall, Joep Welling, Frederic Wigley, Gabriele Valentini, Ulrich A Walker, Francesco Zulian, Ulf Müller-Ladner, EUSTAR Coauthors, Thomas Daikeler, Elisabetta Lanciano, Radim Bečvář, Michal Tomcik, Ewa Gińdzieńska-Sieśkiewicz, Giovanna Cuomo, Michele Iudici, Simona Rednic, Panayiotis G Vlachoyiannopoulos, Roberto Caporali, Patricia E Carreira, Servicio de Reumatología, Srdan Novak, Tünde Minier, Eugene J Kucharz, Armando Gabrielli, Gianluca Moroncini, Paolo Airo', Roger Hesselstrand, Duska Martinovic, Mislav Radić, Daniela Marasovic-Krstulovic, Yolanda Braun-Moscovici, Alexandra Balbir-Gurman, Andrea Lo Monaco, Paola Caramaschi, Jadranka Morović-Vergles, Melanie I Čulo, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Małgorzata Michalska-Jakubus, Matthias F Seidel, Paul Hasler, Klinik Für Rheumatologie, José A Pereira Da Silva, Maria J Salvador, Bojana Stamenkovic, Aleksandra Stankovic, Mohammed Tikly, Lidia P Ananieva, Lorenzo Beretta, Gabriella Szucs, Szilvia Szamosi, Carlos de la Puente Bujidos, Servicio de Reumatología, Øyvind Midtvedt, Anna-Maria Hoffmann-Vold, David Launay, Eric Hachulla, Valeria Riccieri, Ruxandra Ionescu, Daniela Opris, Carina Mihai, Ilka Herrgott, Christian Beyer, Francesca Ingegnoli, Carlos Alberto von Mühlen, Juan José Alegre-Sancho, Emma Beltrán-Catalán, Martin Aringer, Julia Fantana, Nicolai Leuchten, Anne-Kathrin Tausche, Ellen De Langhe, Marie Vanthuyne, Branimir Anic, Marko Barešić, Miroslav Mayer, Maria Üprus, Kati Otsa, Sule Yavuz, Brigitte Granel, Valderilio F Azevedo, Carolina Muller, Sergio A Jimenez, Serghei Popa, Svetlana Agachi, Thierry Zenone, Simon Stebbings, Joanne Dockerty, Alessandra Vacca, Joanna Schollum, Douglas J Veale, Sergio Toloza, Dong Xu, Jacek Olas, Edoardo Rosato, Rosario Foti, Sabine Adler, Diana Dan, Ewa Wiesik-Szewczyk, Marzena Olesińska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Bernard Imbert
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.
Keywords: Cyclophosphamide; Methotrexate; Systemic Sclerosis; Treatment.
Conflict of interest statement
Competing interests: OK-B: consultancies or speakers bureau: AbbVie, Actelion, Bayer, Inventiva, Pfizer, Roche; JA: grant/research support from BMS, Pfizer, Roche/Chugai, Sanofi-Aventis, Actelion; MB: consultant for Actelion; YA: consultant for: Bayer Pharmaceuticals, Actelion, Pfizer, Inventiva, Medac, Servier, Boehringer Ingelheim, Sanofi-Aventis, CSL Behring, Roche; OD: consultant for: 4D Science, Actelion, Active Biotec, Bayer-Schering, Biogen, Biovitrium, BMS, Boehringer Ingelheim Pharma, EpiPharm, Ergonex, GSK, Inventiva, Medac, Novartis, Pfizer, Pharmacyclics, Roche/Genentech, Sanofi/Genzyme, Serodapharm, Sinoxa and United BioSource Corporation; MC: Mundipharm, Actelion, BMS, Horizon, Pfizer, Biogen, Celltrion, Cellgene; LC: consultant for Actelion and Pfizer; CPD: consulting fees from Roche, Actelion, GSK, Bayer Pharmaceuticals; IF: constultant: Bayer, Roche/Chugai; MF: Actelion; DEF: grant/research support from AbbVie, Actelion, Amgen, BMS, NIH, Novartis, Pfizer, Roche/Genentech and consultancy with AbbVie, Actelion, Amgen, BMS, Cytori, Novartis, Pfizer, Roche/Genentech; NH: lecture fees from Actelion, Bayer, Roche; DK: consultancy with Actelion, BMS, Bayer, Covis, Cytori, Genentech/Roche, Gilead, Sanofi-Aventis and grant from NIH/NIAID, NIH/NIAMS, Bayer and BMS; ALH: consultant/speaker/research funding: Actelion, consultant: Apricus; JMvL: honoraria from MSD, BMS, Pfizer, Eli Lilly, Roche; GR: lecturers fees from Bayer, Pfizer, Novartis, Actelion, GSK, BMS and research grants from Actelion; RS: consultant for: Entelligence Program, supported by Actelion; inPractice Rheumatology, grant support: BMS, Bayer; AS: research grant from Actelion; IT: Actelion; UM-L: grant/research support from: EULAR grant, consultant for: Actelion, GSK, Bayer, Medac, Roche/Chugai.
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Source: PubMed