EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

被引：438

作者：

Kowal-Bielecka, O. ^{[1
]}

Landewe, R. ^{[2
]}

Avouac, J. ^{[3
]}

Chwiesko, S. ^{[1
]}

Miniati, I. ^{[4
]}

Czirjak, L. ^{[5
]}

Clements, P. ^{[6
]}

Denton, C. ^{[7
]}

Farge, D. ^{[8
]}

Fligelstone, K. ^{[9
]}

Foeldvari, I. ^{[10
]}

Furst, D. E.

Mueller-Ladner, U. ^{[11
]}

Seibold, J. ^{[12
]}

Silver, R. M. ^{[13
]}

Takehara, K. ^{[14
]}

Toth, B. Garay ^{[15
]}

Tyndall, A. ^{[16
]}

Valentini, G. ^{[17
]}

van den Hoogen, F. ^{[18
]}

Wigley, F. ^{[19
]}

Zulian, F. ^{[20
]}

Matucci-Cerinic, Marco ^{[4
]}

机构：

[1] Med Univ Bialystok, Dept Rheumatol & Internal Med, Bialystok, Poland

[2] Univ Hosp, Dept Rheumatol & Internal Med, Maastricht, Netherlands

[3] RDU, Rheumatol Dept A, Paris, France

[4] Univ Florence, Dept Biomed, Div Rheumatol, I-50134 Florence, Italy

[5] UP, Dept Rheumatol, Pecs, Hungary

[6] Univ Calif Los Angeles, Dept Rheumatol, Los Angeles, CA USA

[7] RFH, Dept Rheumatol, London, England

[8] HSL, Dept Internal Med, Paris, France

[9] FESCA, Scleroderma Assoc, Harlesden, England

[10] Klin Eilbek, Dept Pediat Rheumatol, Hamburg, Germany

[11] JLU, Dept Rheumatol, Bad Nauheim, Germany

[12] UMI, Dept Rheumatol, Ann Arbor, MI USA

[13] MUSC, Dept Rheumatol, Charleston, SC USA

[14] USM, Dept Dermatol, Kanazawa, Ishikawa, Japan

[15] FESCA, Scleroderma Assoc, Budapest, Hungary

[16] Univ Basel, UH, Dept Rheumatol, Basel, Switzerland

[17] SU, Dept Rheumatol, Naples, Italy

[18] Rheumatol Ctr Sint Maartensklin, Nijmegen, Netherlands

[19] JHU, Dept Rheumatol, Baltimore, MD USA

[20] Univ Padua, Dept Pediat, Padua, Italy

来源：

ANNALS OF THE RHEUMATIC DISEASES | 2009年 / 68卷 / 05期

关键词：

PULMONARY-ARTERIAL-HYPERTENSION; DOUBLE-BLIND TRIAL; ENDOTHELIN-RECEPTOR ANTAGONIST; RAYNAUDS-PHENOMENON SECONDARY; ORAL PROSTACYCLIN ANALOG; RENAL CRISIS; INTRAVENOUS EPOPROSTENOL; BACTERIAL OVERGROWTH; SELECTIVE ENDOTHELIN; ILOPROST TREATMENT;

D O I：

10.1136/ard.2008.096677

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：620 / 628

页数：9

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