Update of EULAR recommendations for the treatment of systemic sclerosis
Open accessAbstract
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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According to EULAR recommendations, cyclophosphamide should be considered for treatment of systemic sclerosis-associated interstitial lung disease, in particular for patients with progressive interstitial lung disease, based on two high-quality randomized controlled trials.
“According to EULAR recommendations, 145 in view of the results from two high-quality RCTs 146,147 and despite its known toxicity, CYC should be considered for treatment of SSc-ILD, in particular for patients with SSc with progressive ILD.”
From Autoimmune diseases and immune-checkpoint inhibitors for cancer therapy: Review of the literature and personalized risk-based prevention strategy (2020) · cites this paper as [145]
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Haanen, J., Ernstoff, M. S., Wang, Y., Menzies, A. M., Puzanov, I., Grivas, P., Larkin, J., Peters, S., Thompson, J. A., & Obeid, M. (2020) · Annals of Oncology · doi:10.1016/j.annonc.2020.03.285 · Cited by 222 · see citing works
Unmet medical needs in chronic, non-communicable inflammatory skin diseases
Ujiie, H., Rosmarin, D., Schön, M. P., Ständer, S., Boch, K., Metz, M., Maurer, M., Thaci, D., Schmidt, E., Cole, C., et al. (2022) · Frontiers in Medicine · doi:10.3389/fmed.2022.875492 · Cited by 158 · see citing works
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Self-citation
- Authors
- Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., Distler, O., Clements, P., Cutolo, M., Czirjak, L., Damjanov, N., del Galdo, F., Denton, C. P., Distler, J. H. W., Foeldvari, I., Figelstone, K., Frerix, M., Furst, D. E., Guiducci, S., Hunzelmann, N., Khanna, D., Matucci-Cerinic, M., Herrick, A. L., van den Hoogen, F., van Laar, J. M., Riemekasten, G., Silver, R., Smith, V., Sulli, A., Tarner, I., Tyndall, A., Welling, J., Wigley, F., Valentini, G., Walker, U. A., Zulian, F., Müller-Ladner, U., Daikeler, T., Lanciano, E., Bečvář, R., Tomcik, M., Gińdzieńska-Sieśkiewicz, E., Cuomo, G., Iudici, M., Rednic, S., Vlachoyiannopoulos, P. G., Caporali, R., Carreira, P. E., de Reumatología, S., Novak, S., Minier, T., Kucharz, E. J., Gabrielli, A., Moroncini, G., Airo', P., Hesselstrand, R., Martinovic, D., Radić, M., Marasovic-Krstulovic, D., Braun-Moscovici, Y., Balbir-Gurman, A., Monaco, A. L., Caramaschi, P., Morović-Vergles, J., Čulo, M. I., Henes, J., Santamaria, V. O., Heitmann, S., Krasowska, D., Michalska-Jakubus, M., Seidel, M. F., Hasler, P., Rheumatologie, K. F., Silva, J. A. P. D., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Ananieva, L. P., Beretta, L., Szucs, G., Szamosi, S., Bujidos, C. D. L. P., de Reumatología, S., Midtvedt, Ø., Hoffmann-Vold, A.-M., Launay, D., Hachulla, E., Riccieri, V., Ionescu, R., Opris, D., Mihai, C., Herrgott, I., Beyer, C., Ingegnoli, F., von Mühlen, C. A., Alegre-Sancho, J. J., Beltrán-Catalán, E., Aringer, M., Fantana, J., Leuchten, N., Tausche, A.-K., De Langhe, E., Vanthuyne, M., Anic, B., Barešić, M., Mayer, M., Üprus, M., Otsa, K., Yavuz, S., Granel, B., Azevedo, V. F., Muller, C., Jimenez, S. A., Popa, S., Agachi, S., Zenone, T., Stebbings, S., Dockerty, J., Vacca, A., Schollum, J., Veale, D. J., Toloza, S., Xu, D., Olas, J., Rosato, E., Foti, R., Adler, S., Dan, D., Wiesik-Szewczyk, E., Olesińska, M., Kayser, C., Fathi, N., Lefebvre, P. G. D. L. P., & Imbert, B.
- Affiliations
- Radboud University Medical Center (NL)
- University Hospital Zurich (CH)
- Medical University of Białystok (PL)
- Hôpital Cochin (FR)
- University of California, Los Angeles (US)
- Ospedale Policlinico San Martino (IT)
- University of Belgrade (RS)
- University College London (GB)
- Friedrich-Alexander-Universität Erlangen-Nürnberg (DE)
- Justus-Liebig-Universität Gießen (DE)
- University of Florence (IT)
- University of Cologne (DE)
- University of Michigan (US)
- Manchester Academic Health Science Centre (GB)
- University of Lübeck (DE)
- Medical University of South Carolina (US)
- Ghent University Hospital (BE)
- University of Basel (CH)
- Johns Hopkins University (US)
- University of Campania "Luigi Vanvitelli" (IT)
- University of Padua (IT)
- ORCID
- Fransen, Jaap
- Becker, Mike
- Kulak, Agnieszka
- Allanore, Yannick
- Distler, Oliver
- Cutolo, Maurizio
- Denton, Christopher P
- Distler, Jörg H W
- Foeldvari, Ivan
- Guiducci, Serena
- Khanna, Dinesh
- Herrick, Ariane L
- Riemekasten, Gabriela
- Silver, Richard
- Smith, Vanessa
- Sulli, Alberto
- Tarner, Ingo
- Welling, Joep
- Valentini, Gabriele
- Walker, Ulrich A
- Zulian, Francesco
- Bečvář, Radim
- Cuomo, Giovanna
- Iudici, Michele
- Caporali, Roberto
- Carreira, Patricia E.
- Moroncini, Gianluca
- Radić, Mislav
- Krasowska, Dorota
- Launay, David
- Hachulla, Eric
- Riccieri, Valeria
- Ingegnoli, Francesca
- Aringer, Martin
- Barešić, Marko
- Granel, Brigitte
- Popa, Serghei
- Agachi, Svetlana
- Vacca, Alessandra
- Veale, Douglas J.
- Rosato, Edoardo
- Olesińska, Marzena
- Venue
- Annals of the Rheumatic Diseases
- Publisher
- Elsevier BV
- Published
- 2017-08
- Volume
- 76
- Issue
- 8
- Pages
- 1327-1339
- ISSN
- 0003-4967 (ISSN-L)
- OpenAlex
- W2549071917
- Cited by
- 1,104
- References
- 98
- Citations by year
- 2026: 34 · 2025: 70 · 2024: 152 · 2023: 101 · 2022: 167 · 2021: 170 · 2020: 150 · 2019: 139 · 2018: 80 · 2017: 30 · 2016: 6 · 2015: 2
- FWCI
- 77.13
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Annals of the Rheumatic Diseases (publishedVersion)
- PubMed (publishedVersion)
- Kölner Universitäts PublikationsServer (Universität zu Köln) (publishedVersion)
- Zurich Open Repository and Archive (University of Zurich) (acceptedVersion)
- Dspace Repository (Marmara Üniversitesi) (submittedVersion)
- Istanbul Science University (Istanbul Bilim University) (submittedVersion, other-oa)
- Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion)
- Digital Access to Libraries (Université catholique de Louvain (UCL), l'Université de Namur (UNamur) and the Université Saint-Louis (USL-B)) (submittedVersion, other-oa)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- UCL Discovery (University College London) (submittedVersion)
- University of Debrecen Electronic Archive (University of Debrecen) (submittedVersion)
- Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli" (submittedVersion)
- CINECA IRIS Institutial Research Information System (University of Genoa) (submittedVersion)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion)
- Archivio Istituzionale della Ricerca - Università Vita-Salute San Raffaele (submittedVersion)
- Università Politecnica delle Marche (Università Politecnica delle Marche) (submittedVersion)
- Lirias (KU Leuven) (submittedVersion)
- Lund University Publications (Lund University) (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Dspace Repository (Marmara Üniversitesi) (submittedVersion, other-oa)
- Research Explorer (The University of Manchester) (submittedVersion, other-oa)
- Padua Research Archive (University of Padova) (submittedVersion)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion)
- Zurich Open Repository and Archive (University of Zurich)
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Keywords
- systemic sclerosis, scleroderma treatment, EULAR recommendations, Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, phosphodiesterase-5 inhibitors, riociguat, endothelin receptor antagonists, prostacyclin analogues, scleroderma renal crisis, gastrointestinal involvement, hematopoietic stem cell transplantation, fluoxetine
- MeSH terms
- Angiotensin-Converting Enzyme Inhibitors, Delphi Technique, Europe, Fingers, Fluoxetine, Gastrointestinal Diseases, Glucocorticoids, Humans, Hypertension, Pulmonary, Kidney Diseases, Lung Diseases, Pyrazoles, Pyrimidines, Raynaud Disease, Rheumatology, Scleroderma, Systemic, Ulcer, Selective Serotonin Reuptake Inhibitors, Hematopoietic Stem Cell Transplantation, Prostaglandins I, Phosphodiesterase 5 Inhibitors, Endothelin Receptor Antagonists
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