Kowal-Bielecka, Otylia ORCID: 0000-0002-0613-1306, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver ORCID: 0000-0002-0546-8310, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Joerg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena ORCID: 0000-0003-2722-6475, Hunzelmann, Nicolas, Khanna, Dinesh, Matucci-Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank ORCID: 0000-0003-1044-015X, 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 and Mueller-Ladner, Ulf (2017). Update of EULAR recommendations for the treatment of systemic sclerosis. Ann. Rheum. Dis., 76 (8). S. 1327 - 1340. LONDON: BMJ PUBLISHING GROUP. ISSN 1468-2060

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Kowal-Bielecka, OtyliaUNSPECIFIEDorcid.org/0000-0002-0613-1306UNSPECIFIED
Fransen, JaapUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Avouac, JeromeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Becker, MikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kulak, AgnieszkaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Allanore, YannickUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Distler, OliverUNSPECIFIEDorcid.org/0000-0002-0546-8310UNSPECIFIED
Clements, PhilipUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cutolo, MaurizioUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Czirjak, LaszloUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Damjanov, NemanjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
del Galdo, FrancescoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Denton, Christopher P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Distler, Joerg H. W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Foeldvari, IvanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Figelstone, KimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Frerix, MarcUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Furst, Daniel E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Guiducci, SerenaUNSPECIFIEDorcid.org/0000-0003-2722-6475UNSPECIFIED
Hunzelmann, NicolasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Khanna, DineshUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Matucci-Cerinic, MarcoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Herrick, Ariane L.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van den Hoogen, FrankUNSPECIFIEDorcid.org/0000-0003-1044-015XUNSPECIFIED
van Laar, Jacob M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Riemekasten, GabrielaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Silver, RichardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Smith, VanessaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sulli, AlbertoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tarner, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tyndall, AlanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Welling, JoepUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wigley, FredericUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valentini, GabrieleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Walker, Ulrich A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zulian, FrancescoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mueller-Ladner, UlfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-223573
DOI: 10.1136/annrheumdis-2016-209909
Journal or Publication Title: Ann. Rheum. Dis.
Volume: 76
Number: 8
Page Range: S. 1327 - 1340
Date: 2017
Publisher: BMJ PUBLISHING GROUP
Place of Publication: LONDON
ISSN: 1468-2060
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
SCLERODERMA RENAL CRISIS; INTESTINAL BACTERIAL OVERGROWTH; DOUBLE-BLIND TRIAL; STANDARDIZED OPERATING PROCEDURES; PULMONARY ARTERIAL-HYPERTENSION; RAYNAUDS-PHENOMENON SECONDARY; STEM-CELL TRANSPLANTATION; ORAL PROSTACYCLIN ANALOG; DIGITAL ULCERS; INTRAVENOUS EPOPROSTENOLMultiple languages
RheumatologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/22357

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