EULAR recommendations for the treatment of systemic sclerosis

A report from the EULAR Scleroderma Trials and Research group (EUSTAR)

O. Kowal-Bielecka, R. Landewé, J. Avouac, S. Chwiesko, I. Miniati, L. Czirjak, P. Clements, C. Denton, D. Farge, K. Fligelstone, I. Földvari, D. E. Furst, U. Müller-Ladner, J. Seibold, R. M. Silver, K. Takehara, B. Garay Toth, A. Tyndall, G. Valentini, F. Van Den Hoogen & 75 others F. Wigley, F. Zulian, Marco Matucci-Cerinic, F. Wollheim, A. Scheja, T. Nevskaya, L. Ananieva, E. Nassonov, Y. Allanore, A. Kahan, E. Hachulla, D. Launay, A. Herrick, J. Van Laar, C. Chizzolini, C. Ciurtin, D. Opris, R. Ionescu, M. Capraru, S. Rednic, O. Distler, I. Simsek, A. Dinc, M. Inanc, W. Graninger, I. Kotter, J. T. Gran, O. Midvedt, E. Kucharz, D. Krasowska, M. Majdan, S. Sierakowski, M. J. Salvador, J. A. Pereira Da Silva, N. Damjanov, P. Carreira, P. Garcia De La Pena Lefebvre, G. Szucs, Z. Szekanecz, R. Becvar, P. Pinto, P. Coelho, S. Novak, U. Walker, M. Aringer, T. Glaenz, C. H. Neumann, S. Emmert, M. Worm, G. Riemekasten, T. Krieg, N. Hunzelmann, K. Steinbrink, C. Sunderkotter, S. Heitmann, I. Foldvari, F. Iannone, G. Lapadula, P. Airň, R. Giacomelli, R. Scorza, M. Rizzi, F. Indiveri, V. Riccieri, F. Zulian, B. Rozman, K. Takehara, Jae Bum Jun, C. Derk, N. Sandorfi, S. Jimenez, M. Tikly, Saad Alhasani, Y. Shoenfeld, A. Balbir Gurmann

Research output: Contribution to journalArticle

446 Citations (Scopus)

Abstract

Purpose: The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc. Methods: To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres. Results: Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud's phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda. Conclusions: Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

Original languageEnglish
Pages (from-to)620-628
Number of pages9
JournalAnnals of the Rheumatic Diseases
Volume68
Issue number5
DOIs
Publication statusPublished - May 2009

Fingerprint

Systemic Scleroderma
Rheumatic Diseases
Research
Therapeutics
Pulmonary diseases
Skin
Raynaud Disease
Interstitial Lung Diseases
Expert Testimony
Advisory Committees
Pulmonary Hypertension
Ulcer
Japan

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Immunology and Allergy

Cite this

Kowal-Bielecka, O., Landewé, R., Avouac, J., Chwiesko, S., Miniati, I., Czirjak, L., ... Balbir Gurmann, A. (2009). EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Annals of the Rheumatic Diseases, 68(5), 620-628. https://doi.org/10.1136/ard.2008.096677

EULAR recommendations for the treatment of systemic sclerosis : A report from the EULAR Scleroderma Trials and Research group (EUSTAR). / Kowal-Bielecka, O.; Landewé, R.; Avouac, J.; Chwiesko, S.; Miniati, I.; Czirjak, L.; Clements, P.; Denton, C.; Farge, D.; Fligelstone, K.; Földvari, I.; Furst, D. E.; Müller-Ladner, U.; Seibold, J.; Silver, R. M.; Takehara, K.; Garay Toth, B.; Tyndall, A.; Valentini, G.; Van Den Hoogen, F.; Wigley, F.; Zulian, F.; Matucci-Cerinic, Marco; Wollheim, F.; Scheja, A.; Nevskaya, T.; Ananieva, L.; Nassonov, E.; Allanore, Y.; Kahan, A.; Hachulla, E.; Launay, D.; Herrick, A.; Van Laar, J.; Chizzolini, C.; Ciurtin, C.; Opris, D.; Ionescu, R.; Capraru, M.; Rednic, S.; Distler, O.; Simsek, I.; Dinc, A.; Inanc, M.; Graninger, W.; Kotter, I.; Gran, J. T.; Midvedt, O.; Kucharz, E.; Krasowska, D.; Majdan, M.; Sierakowski, S.; Salvador, M. J.; Pereira Da Silva, J. A.; Damjanov, N.; Carreira, P.; Garcia De La Pena Lefebvre, P.; Szucs, G.; Szekanecz, Z.; Becvar, R.; Pinto, P.; Coelho, P.; Novak, S.; Walker, U.; Aringer, M.; Glaenz, T.; Neumann, C. H.; Emmert, S.; Worm, M.; Riemekasten, G.; Krieg, T.; Hunzelmann, N.; Steinbrink, K.; Sunderkotter, C.; Heitmann, S.; Foldvari, I.; Iannone, F.; Lapadula, G.; Airň, P.; Giacomelli, R.; Scorza, R.; Rizzi, M.; Indiveri, F.; Riccieri, V.; Zulian, F.; Rozman, B.; Takehara, K.; Jun, Jae Bum; Derk, C.; Sandorfi, N.; Jimenez, S.; Tikly, M.; Alhasani, Saad; Shoenfeld, Y.; Balbir Gurmann, A.

In: Annals of the Rheumatic Diseases, Vol. 68, No. 5, 05.2009, p. 620-628.

Research output: Contribution to journalArticle

Kowal-Bielecka, O, Landewé, R, Avouac, J, Chwiesko, S, Miniati, I, Czirjak, L, Clements, P, Denton, C, Farge, D, Fligelstone, K, Földvari, I, Furst, DE, Müller-Ladner, U, Seibold, J, Silver, RM, Takehara, K, Garay Toth, B, Tyndall, A, Valentini, G, Van Den Hoogen, F, Wigley, F, Zulian, F, Matucci-Cerinic, M, Wollheim, F, Scheja, A, Nevskaya, T, Ananieva, L, Nassonov, E, Allanore, Y, Kahan, A, Hachulla, E, Launay, D, Herrick, A, Van Laar, J, Chizzolini, C, Ciurtin, C, Opris, D, Ionescu, R, Capraru, M, Rednic, S, Distler, O, Simsek, I, Dinc, A, Inanc, M, Graninger, W, Kotter, I, Gran, JT, Midvedt, O, Kucharz, E, Krasowska, D, Majdan, M, Sierakowski, S, Salvador, MJ, Pereira Da Silva, JA, Damjanov, N, Carreira, P, Garcia De La Pena Lefebvre, P, Szucs, G, Szekanecz, Z, Becvar, R, Pinto, P, Coelho, P, Novak, S, Walker, U, Aringer, M, Glaenz, T, Neumann, CH, Emmert, S, Worm, M, Riemekasten, G, Krieg, T, Hunzelmann, N, Steinbrink, K, Sunderkotter, C, Heitmann, S, Foldvari, I, Iannone, F, Lapadula, G, Airň, P, Giacomelli, R, Scorza, R, Rizzi, M, Indiveri, F, Riccieri, V, Zulian, F, Rozman, B, Takehara, K, Jun, JB, Derk, C, Sandorfi, N, Jimenez, S, Tikly, M, Alhasani, S, Shoenfeld, Y & Balbir Gurmann, A 2009, 'EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR)', Annals of the Rheumatic Diseases, vol. 68, no. 5, pp. 620-628. https://doi.org/10.1136/ard.2008.096677
Kowal-Bielecka, O. ; Landewé, R. ; Avouac, J. ; Chwiesko, S. ; Miniati, I. ; Czirjak, L. ; Clements, P. ; Denton, C. ; Farge, D. ; Fligelstone, K. ; Földvari, I. ; Furst, D. E. ; Müller-Ladner, U. ; Seibold, J. ; Silver, R. M. ; Takehara, K. ; Garay Toth, B. ; Tyndall, A. ; Valentini, G. ; Van Den Hoogen, F. ; Wigley, F. ; Zulian, F. ; Matucci-Cerinic, Marco ; Wollheim, F. ; Scheja, A. ; Nevskaya, T. ; Ananieva, L. ; Nassonov, E. ; Allanore, Y. ; Kahan, A. ; Hachulla, E. ; Launay, D. ; Herrick, A. ; Van Laar, J. ; Chizzolini, C. ; Ciurtin, C. ; Opris, D. ; Ionescu, R. ; Capraru, M. ; Rednic, S. ; Distler, O. ; Simsek, I. ; Dinc, A. ; Inanc, M. ; Graninger, W. ; Kotter, I. ; Gran, J. T. ; Midvedt, O. ; Kucharz, E. ; Krasowska, D. ; Majdan, M. ; Sierakowski, S. ; Salvador, M. J. ; Pereira Da Silva, J. A. ; Damjanov, N. ; Carreira, P. ; Garcia De La Pena Lefebvre, P. ; Szucs, G. ; Szekanecz, Z. ; Becvar, R. ; Pinto, P. ; Coelho, P. ; Novak, S. ; Walker, U. ; Aringer, M. ; Glaenz, T. ; Neumann, C. H. ; Emmert, S. ; Worm, M. ; Riemekasten, G. ; Krieg, T. ; Hunzelmann, N. ; Steinbrink, K. ; Sunderkotter, C. ; Heitmann, S. ; Foldvari, I. ; Iannone, F. ; Lapadula, G. ; Airň, P. ; Giacomelli, R. ; Scorza, R. ; Rizzi, M. ; Indiveri, F. ; Riccieri, V. ; Zulian, F. ; Rozman, B. ; Takehara, K. ; Jun, Jae Bum ; Derk, C. ; Sandorfi, N. ; Jimenez, S. ; Tikly, M. ; Alhasani, Saad ; Shoenfeld, Y. ; Balbir Gurmann, A. / EULAR recommendations for the treatment of systemic sclerosis : A report from the EULAR Scleroderma Trials and Research group (EUSTAR). In: Annals of the Rheumatic Diseases. 2009 ; Vol. 68, No. 5. pp. 620-628.
@article{ed2a3e1ff8294fb4bbe04db72b7ddac4,
title = "EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR)",
abstract = "Purpose: The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc. Methods: To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres. Results: Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud's phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda. Conclusions: Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.",
author = "O. Kowal-Bielecka and R. Landew{\'e} and J. Avouac and S. Chwiesko and I. Miniati and L. Czirjak and P. Clements and C. Denton and D. Farge and K. Fligelstone and I. F{\"o}ldvari and Furst, {D. E.} and U. M{\"u}ller-Ladner and J. Seibold and Silver, {R. M.} and K. Takehara and {Garay Toth}, B. and A. Tyndall and G. Valentini and {Van Den Hoogen}, F. and F. Wigley and F. Zulian and Marco Matucci-Cerinic and F. Wollheim and A. Scheja and T. Nevskaya and L. Ananieva and E. Nassonov and Y. Allanore and A. Kahan and E. Hachulla and D. Launay and A. Herrick and {Van Laar}, J. and C. Chizzolini and C. Ciurtin and D. Opris and R. Ionescu and M. Capraru and S. Rednic and O. Distler and I. Simsek and A. Dinc and M. Inanc and W. Graninger and I. Kotter and Gran, {J. T.} and O. Midvedt and E. Kucharz and D. Krasowska and M. Majdan and S. Sierakowski and Salvador, {M. J.} and {Pereira Da Silva}, {J. A.} and N. Damjanov and P. Carreira and {Garcia De La Pena Lefebvre}, P. and G. Szucs and Z. Szekanecz and R. Becvar and P. Pinto and P. Coelho and S. Novak and U. Walker and M. Aringer and T. Glaenz and Neumann, {C. H.} and S. Emmert and M. Worm and G. Riemekasten and T. Krieg and N. Hunzelmann and K. Steinbrink and C. Sunderkotter and S. Heitmann and I. Foldvari and F. Iannone and G. Lapadula and P. Airň and R. Giacomelli and R. Scorza and M. Rizzi and F. Indiveri and V. Riccieri and F. Zulian and B. Rozman and K. Takehara and Jun, {Jae Bum} and C. Derk and N. Sandorfi and S. Jimenez and M. Tikly and Saad Alhasani and Y. Shoenfeld and {Balbir Gurmann}, A.",
year = "2009",
month = "5",
doi = "10.1136/ard.2008.096677",
language = "English",
volume = "68",
pages = "620--628",
journal = "Annals of the Rheumatic Diseases",
issn = "0003-4967",
publisher = "BMJ Publishing Group",
number = "5",

}

TY - JOUR

T1 - EULAR recommendations for the treatment of systemic sclerosis

T2 - A report from the EULAR Scleroderma Trials and Research group (EUSTAR)

AU - Kowal-Bielecka, O.

AU - Landewé, R.

AU - Avouac, J.

AU - Chwiesko, S.

AU - Miniati, I.

AU - Czirjak, L.

AU - Clements, P.

AU - Denton, C.

AU - Farge, D.

AU - Fligelstone, K.

AU - Földvari, I.

AU - Furst, D. E.

AU - Müller-Ladner, U.

AU - Seibold, J.

AU - Silver, R. M.

AU - Takehara, K.

AU - Garay Toth, B.

AU - Tyndall, A.

AU - Valentini, G.

AU - Van Den Hoogen, F.

AU - Wigley, F.

AU - Zulian, F.

AU - Matucci-Cerinic, Marco

AU - Wollheim, F.

AU - Scheja, A.

AU - Nevskaya, T.

AU - Ananieva, L.

AU - Nassonov, E.

AU - Allanore, Y.

AU - Kahan, A.

AU - Hachulla, E.

AU - Launay, D.

AU - Herrick, A.

AU - Van Laar, J.

AU - Chizzolini, C.

AU - Ciurtin, C.

AU - Opris, D.

AU - Ionescu, R.

AU - Capraru, M.

AU - Rednic, S.

AU - Distler, O.

AU - Simsek, I.

AU - Dinc, A.

AU - Inanc, M.

AU - Graninger, W.

AU - Kotter, I.

AU - Gran, J. T.

AU - Midvedt, O.

AU - Kucharz, E.

AU - Krasowska, D.

AU - Majdan, M.

AU - Sierakowski, S.

AU - Salvador, M. J.

AU - Pereira Da Silva, J. A.

AU - Damjanov, N.

AU - Carreira, P.

AU - Garcia De La Pena Lefebvre, P.

AU - Szucs, G.

AU - Szekanecz, Z.

AU - Becvar, R.

AU - Pinto, P.

AU - Coelho, P.

AU - Novak, S.

AU - Walker, U.

AU - Aringer, M.

AU - Glaenz, T.

AU - Neumann, C. H.

AU - Emmert, S.

AU - Worm, M.

AU - Riemekasten, G.

AU - Krieg, T.

AU - Hunzelmann, N.

AU - Steinbrink, K.

AU - Sunderkotter, C.

AU - Heitmann, S.

AU - Foldvari, I.

AU - Iannone, F.

AU - Lapadula, G.

AU - Airň, P.

AU - Giacomelli, R.

AU - Scorza, R.

AU - Rizzi, M.

AU - Indiveri, F.

AU - Riccieri, V.

AU - Zulian, F.

AU - Rozman, B.

AU - Takehara, K.

AU - Jun, Jae Bum

AU - Derk, C.

AU - Sandorfi, N.

AU - Jimenez, S.

AU - Tikly, M.

AU - Alhasani, Saad

AU - Shoenfeld, Y.

AU - Balbir Gurmann, A.

PY - 2009/5

Y1 - 2009/5

N2 - Purpose: The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc. Methods: To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres. Results: Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud's phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda. Conclusions: Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

AB - Purpose: The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group (EUSTAR) was to develop evidence-based, consensus-derived recommendations for the treatment of SSc. Methods: To obtain and maintain a high level of intrinsic quality and comparability of this approach, EULAR standard operating procedures were followed. The task force comprised 18 SSc experts from Europe, the USA and Japan, two SSc patients and three fellows for literature research. The preliminary set of research questions concerning SSc treatment was provided by 74 EUSTAR centres. Results: Based on discussion of the clinical research evidence from published literature, and combining this with current expert opinion and clinical experience, 14 recommendations for the treatment of SSc were formulated. The final set includes the following recommendations: three on SSc-related digital vasculopathy (Raynaud's phenomenon and ulcers); four on SSc-related pulmonary arterial hypertension; three on SSc-related gastrointestinal involvement; two on scleroderma renal crisis; one on SSc-related interstitial lung disease and one on skin involvement. Experts also formulated several questions for a future research agenda. Conclusions: Evidence-based, consensus-derived recommendations are useful for rheumatologists to help guide treatment for patients with SSc. These recommendations may also help to define directions for future clinical research in SSc.

UR - http://www.scopus.com/inward/record.url?scp=66149100618&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=66149100618&partnerID=8YFLogxK

U2 - 10.1136/ard.2008.096677

DO - 10.1136/ard.2008.096677

M3 - Article

VL - 68

SP - 620

EP - 628

JO - Annals of the Rheumatic Diseases

JF - Annals of the Rheumatic Diseases

SN - 0003-4967

IS - 5

ER -