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Update of EULAR recommendations for the treatment of systemic sclerosis

(2016) ANNALS OF THE RHEUMATIC DISEASES. 76(8). p.1327-1339
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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.
Keywords
SCLERODERMA RENAL CRISIS, PULMONARY ARTERIAL-HYPERTENSION, DOUBLE-BLIND TRIAL, INTESTINAL BACTERIAL OVERGROWTH, STANDARDIZED OPERATING PROCEDURES, RAYNAUDS-PHENOMENON SECONDARY, STEM-CELL TRANSPLANTATION, INTERSTITIAL LUNG-DISEASE, ORAL PROSTACYCLIN ANALOG, PLACEBO-CONTROLLED TRIAL

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Citation

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Chicago
Kowal-Bielecka, Otylia, Jaap Fransen, Jerome Avouac, Mike Becker, Agnieszka Kulak, Yannick Allanore, Oliver Distler, et al. 2016. “Update of EULAR Recommendations for the Treatment of Systemic Sclerosis.” Annals of the Rheumatic Diseases 76 (8): 1327–1339.
APA
Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., Distler, O., et al. (2016). Update of EULAR recommendations for the treatment of systemic sclerosis. ANNALS OF THE RHEUMATIC DISEASES, 76(8), 1327–1339.
Vancouver
1.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. ANNALS OF THE RHEUMATIC DISEASES. 2016;76(8):1327–39.
MLA
Kowal-Bielecka, Otylia et al. “Update of EULAR Recommendations for the Treatment of Systemic Sclerosis.” ANNALS OF THE RHEUMATIC DISEASES 76.8 (2016): 1327–1339. Print.
@article{8543875,
  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.},
  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 HW 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},
  issn         = {0003-4967},
  journal      = {ANNALS OF THE RHEUMATIC DISEASES},
  keywords     = {SCLERODERMA RENAL CRISIS,PULMONARY ARTERIAL-HYPERTENSION,DOUBLE-BLIND TRIAL,INTESTINAL BACTERIAL OVERGROWTH,STANDARDIZED OPERATING PROCEDURES,RAYNAUDS-PHENOMENON SECONDARY,STEM-CELL TRANSPLANTATION,INTERSTITIAL LUNG-DISEASE,ORAL PROSTACYCLIN ANALOG,PLACEBO-CONTROLLED TRIAL},
  language     = {eng},
  number       = {8},
  pages        = {1327--1339},
  title        = {Update of EULAR recommendations for the treatment of systemic sclerosis},
  url          = {http://dx.doi.org/10.1136/annrheumdis-2016-209909},
  volume       = {76},
  year         = {2016},
}

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