Update of EULAR recommendations for the treatment of systemic sclerosis

被引：832

作者：

Kowal-Bielecka, Otylia ^{[1
]}

Fransen, Jaap ^{[2
]}

Avouac, Jerome ^{[3
]}

Becker, Mike ^{[4
,5
]}

Kulak, Agnieszka ^{[1
]}

Allanore, Yannick ^{[3
]}

Distler, Oliver ^{[5
]}

Clements, Philip ^{[6
]}

Cutolo, Maurizio ^{[7
,8
]}

Czirjak, Laszlo ^{[9
]}

Damjanov, Nemanja ^{[10
]}

del Galdo, Francesco ^{[11
]}

Denton, Christopher P. ^{[12
]}

Distler, Joerg H. W. ^{[13
]}

Foeldvari, Ivan ^{[14
]}

Figelstone, Kim ^{[15
]}

Frerix, Marc ^{[16
]}

Furst, Daniel E. ^{[6
]}

Guiducci, Serena ^{[17
]}

Hunzelmann, Nicolas ^{[18
]}

Khanna, Dinesh ^{[19
]}

Matucci-Cerinic, Marco ^{[17
]}

Herrick, Ariane L. ^{[20
,21
]}

van den Hoogen, Frank ^{[2
]}

van Laar, Jacob M. ^{[22
]}

Riemekasten, Gabriela ^{[23
]}

Silver, Richard ^{[24
]}

Smith, Vanessa ^{[25
]}

Sulli, Alberto ^{[7
,8
]}

Tarner, Ingo ^{[16
]}

Tyndall, Alan ^{[26
]}

Welling, Joep ^{[27
]}

Wigley, Frederic ^{[28
]}

Valentini, Gabriele ^{[29
]}

Walker, Ulrich A. ^{[26
]}

Zulian, Francesco ^{[30
]}

Mueller-Ladner, Ulf ^{[16
]}

机构：

[1] Med Univ Bialystok, Dept Rheumatol & Internal Med, Ul M Sklodowskiej Curie 24A, PL-15276 Bialystok, Poland

[2] Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands

[3] Paris Descartes Univ, Cochin Hosp, Rheumatol Dept A, Paris, France

[4] Univ Hosp Charite, Berlin, Germany

[5] Univ Hosp Zurich, Zurich, Switzerland

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

[7] Univ Genoa, IRCCS AOU San Martino, Res Labs, Genoa, Italy

[8] Univ Genoa, IRCCS AOU San Martino, Clin Div Rheumatol, Dept Internal Med, Genoa, Italy

[9] Univ Pecs, Dept Rheumatol & Immunol, Med Ctr, Pecs, Hungary

[10] Univ Belgrade, Belgrade, Serbia

[11] Univ Leeds, Leeds, W Yorkshire, England

[12] UCL, London, England

[13] Univ Erlangen Nurnberg, Erlangen, Germany

[14] Hamburg Ctr Pediat & Adolescence Rheumatol, Hamburg, Germany

[15] FESCA, London, England

[16] Univ Giessen, Bad Nauheim, Germany

[17] Univ Florence, Florence, Italy

[18] Univ Cologne, Cologne, Germany

[19] Univ Michigan, Sch Med, Ann Arbor, MI USA

[20] Univ Manchester, Salford Royal NHS Fdn Trust, Manchester Acad Hlth Sci Ctr, Manchester, Lancs, England

[21] Cent Manchester NHS Fdn Trust, NIHR Manchester Musculoskeletal Biomed Res Unit, Manchester, Lancs, England

[22] Univ Med Ctr Utrecht, Dept Rheumatol & Clin Immunol, Utrecht, Netherlands

[23] Univ Lubeck, Lubeck, Germany

[24] Med Univ South Carolina, Charleston, SC USA

[25] Univ Ghent, Ghent Univ Hosp, Ghent, Belgium

[26] Basel Univ, Basel, Switzerland

[27] FESCA Patient Res Partner, Ede, Netherlands

[28] Johns Hopkins Univ, Baltimore, MD USA

[29] Univ Naples 2, Naples, Italy

[30] Univ Padua, Padua, Italy

来源：

ANNALS OF THE RHEUMATIC DISEASES | 2017年 / 76卷 / 08期

关键词：

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 EPOPROSTENOL;

D O I：

10.1136/annrheumdis-2016-209909

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1327 / 1339

页数：13

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