Update of EULAR recommendations for the treatment of systemic sclerosis

O. Kowal-Bielecka, J. Fransen, J. Avouac, M. Becker, A. Kulak, Y. Allanore, O. Distler, P. Clements, M. Cutolo, L. Czirjak, N. Damjanov, F. Del Galdo, C. P. Denton, J. H. W. Distler, I. Foeldvari, K. Figelstone, M. Frerix, D. E. Furst, S. Guiducci, N. HunzelmannD. Khanna, M. Matucci-Cerinic, A. L. Herrick, F. van den Hoogen, J. M. van Laar, G. Riemekasten, R. Silver, V. Smith, A. Sulli, I. Tarner, A. Tyndall, J. Welling, F. Wigley, G. Valentini, U. A. Walker, F. Zulian, U. Muller-Ladner, EUSTAR Coauthors

Research output: Contribution to journalArticle

185 Citations (Scopus)

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.
Original languageEnglish
Pages (from-to)1327-1339
Number of pages13
JournalAnnals of the Rheumatic Diseases
Volume76
Issue number8
DOIs
Publication statusPublished - Aug 1 2017
Externally publishedYes

Fingerprint

Systemic Scleroderma
Rheumatic Diseases
Phosphodiesterase 5 Inhibitors
Raynaud Disease
Pulmonary diseases
Fluoxetine
Epoprostenol
Therapeutics
Stem cells
Skin
Research
Pulmonary Hypertension
Ulcer
Diffuse Scleroderma
Hematopoietic Stem Cell Transplantation
Advisory Committees
Skin Diseases
Lung Diseases
Kidney

Keywords

  • Angiotensin-Converting Enzyme Inhibitors/therapeutic use
  • Delphi Technique
  • Endothelin Receptor Antagonists/therapeutic use
  • Europe
  • Fingers
  • Fluoxetine/therapeutic use
  • Gastrointestinal Diseases/etiology/therapy
  • Glucocorticoids/therapeutic use
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Hypertension, Pulmonary/etiology/therapy
  • Kidney Diseases/etiology/therapy
  • Lung Diseases/etiology/therapy
  • Phosphodiesterase 5 Inhibitors/therapeutic use
  • Prostaglandins I/therapeutic use
  • Pyrazoles/therapeutic use
  • Pyrimidines/therapeutic use
  • Raynaud Disease/etiology/therapy
  • Rheumatology
  • Scleroderma, Systemic/complications/therapy
  • Serotonin Uptake Inhibitors/therapeutic use
  • Ulcer/etiology/therapy
  • Cyclophosphamide
  • Methotrexate
  • Systemic Sclerosis
  • Treatment

Cite this

Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., ... Coauthors, EUSTAR. (2017). Update of EULAR recommendations for the treatment of systemic sclerosis. Annals of the Rheumatic Diseases, 76(8), 1327-1339. https://doi.org/10.1136/annrheumdis-2016-209909 [doi]

Update of EULAR recommendations for the treatment of systemic sclerosis. / Kowal-Bielecka, O.; Fransen, J.; Avouac, J.; Becker, M.; Kulak, A.; Allanore, Y.; Distler, O.; Clements, P.; Cutolo, M.; Czirjak, L.; Damjanov, N.; Galdo, F. Del; 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.; Hoogen, F. van den; Laar, J. M. van; Riemekasten, G.; Silver, R.; Smith, V.; Sulli, A.; Tarner, I.; Tyndall, A.; Welling, J.; Wigley, F.; Valentini, G.; Walker, U. A.; Zulian, F.; Muller-Ladner, U.; Coauthors, EUSTAR.

In: Annals of the Rheumatic Diseases, Vol. 76, No. 8, 01.08.2017, p. 1327-1339.

Research output: Contribution to journalArticle

Kowal-Bielecka, O, Fransen, J, Avouac, J, Becker, M, Kulak, A, Allanore, Y, Distler, O, Clements, P, Cutolo, M, Czirjak, L, Damjanov, N, Galdo, FD, Denton, CP, Distler, JHW, Foeldvari, I, Figelstone, K, Frerix, M, Furst, DE, Guiducci, S, Hunzelmann, N, Khanna, D, Matucci-Cerinic, M, Herrick, AL, Hoogen, FVD, Laar, JMV, Riemekasten, G, Silver, R, Smith, V, Sulli, A, Tarner, I, Tyndall, A, Welling, J, Wigley, F, Valentini, G, Walker, UA, Zulian, F, Muller-Ladner, U & Coauthors, EUSTAR 2017, 'Update of EULAR recommendations for the treatment of systemic sclerosis', Annals of the Rheumatic Diseases, vol. 76, no. 8, pp. 1327-1339. https://doi.org/10.1136/annrheumdis-2016-209909 [doi]
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. 2017 Aug 1;76(8):1327-1339. https://doi.org/10.1136/annrheumdis-2016-209909 [doi]
Kowal-Bielecka, O. ; Fransen, J. ; Avouac, J. ; Becker, M. ; Kulak, A. ; Allanore, Y. ; Distler, O. ; Clements, P. ; Cutolo, M. ; Czirjak, L. ; Damjanov, N. ; Galdo, F. Del ; 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. ; Hoogen, F. van den ; Laar, J. M. van ; Riemekasten, G. ; Silver, R. ; Smith, V. ; Sulli, A. ; Tarner, I. ; Tyndall, A. ; Welling, J. ; Wigley, F. ; Valentini, G. ; Walker, U. A. ; Zulian, F. ; Muller-Ladner, U. ; Coauthors, EUSTAR. / Update of EULAR recommendations for the treatment of systemic sclerosis. In: Annals of the Rheumatic Diseases. 2017 ; Vol. 76, No. 8. pp. 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.",
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TY - JOUR

T1 - Update of EULAR recommendations for the treatment of systemic sclerosis

AU - Kowal-Bielecka, O.

AU - Fransen, J.

AU - Avouac, J.

AU - Becker, M.

AU - Kulak, A.

AU - Allanore, Y.

AU - Distler, O.

AU - Clements, P.

AU - Cutolo, M.

AU - Czirjak, L.

AU - Damjanov, N.

AU - Galdo, F. Del

AU - Denton, C. P.

AU - Distler, J. H. W.

AU - Foeldvari, I.

AU - Figelstone, K.

AU - Frerix, M.

AU - Furst, D. E.

AU - Guiducci, S.

AU - Hunzelmann, N.

AU - Khanna, D.

AU - Matucci-Cerinic, M.

AU - Herrick, A. L.

AU - Hoogen, F. van den

AU - Laar, J. M. van

AU - Riemekasten, G.

AU - Silver, R.

AU - Smith, V.

AU - Sulli, A.

AU - Tarner, I.

AU - Tyndall, A.

AU - Welling, J.

AU - Wigley, F.

AU - Valentini, G.

AU - Walker, U. A.

AU - Zulian, F.

AU - Muller-Ladner, U.

AU - Coauthors, EUSTAR

N1 - LR: 20171116; CI: Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.; JID: 0372355; 0 (Angiotensin-Converting Enzyme Inhibitors); 0 (Endothelin Receptor Antagonists); 0 (Glucocorticoids); 0 (Phosphodiesterase 5 Inhibitors); 0 (Prostaglandins I); 0 (Pyrazoles); 0 (Pyrimidines); 0 (Serotonin Uptake Inhibitors); 01K63SUP8D (Fluoxetine); RU3FE2Y4XI (riociguat); OTO: NOTNLM; 2016/05/18 00:00 [received]; 2016/08/22 00:00 [revised]; 2016/10/09 00:00 [accepted]; 2016/12/13 06:00 [pubmed]; 2017/08/22 06:00 [medline]; 2016/12/13 06:00 [entrez]; ppublish

PY - 2017/8/1

Y1 - 2017/8/1

N2 - 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.

AB - 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.

KW - Angiotensin-Converting Enzyme Inhibitors/therapeutic use

KW - Delphi Technique

KW - Endothelin Receptor Antagonists/therapeutic use

KW - Europe

KW - Fingers

KW - Fluoxetine/therapeutic use

KW - Gastrointestinal Diseases/etiology/therapy

KW - Glucocorticoids/therapeutic use

KW - Hematopoietic Stem Cell Transplantation

KW - Humans

KW - Hypertension, Pulmonary/etiology/therapy

KW - Kidney Diseases/etiology/therapy

KW - Lung Diseases/etiology/therapy

KW - Phosphodiesterase 5 Inhibitors/therapeutic use

KW - Prostaglandins I/therapeutic use

KW - Pyrazoles/therapeutic use

KW - Pyrimidines/therapeutic use

KW - Raynaud Disease/etiology/therapy

KW - Rheumatology

KW - Scleroderma, Systemic/complications/therapy

KW - Serotonin Uptake Inhibitors/therapeutic use

KW - Ulcer/etiology/therapy

KW - Cyclophosphamide

KW - Methotrexate

KW - Systemic Sclerosis

KW - Treatment

U2 - 10.1136/annrheumdis-2016-209909 [doi]

DO - 10.1136/annrheumdis-2016-209909 [doi]

M3 - Article

VL - 76

SP - 1327

EP - 1339

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

SN - 0003-4967

IS - 8

ER -