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Research ArticleAccepted Article

Worldwide expert agreement on updated recommendations for the treatment of systemic sclerosis

Jeska de Vries-Bouwstra, Yannick Allanore, Marco Matucci-Cerinic and Alexandra Balbir-Gurman
The Journal of Rheumatology May 2019, jrheum.181173; DOI: https://doi.org/10.3899/jrheum.181173
Jeska de Vries-Bouwstra
From the Leiden University Medical Center, department of Rheumatology, Leiden, The Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology dpt, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, RappaportFaculty of Medicine - Tehnion, Haifa, Israel. Address correspondence to J.K. de Vries-Bouwstra,; Department of Rheumatology, C01-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands; Email: j.k.de_vries-bouwstra@lumc.nl.
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Yannick Allanore
From the Leiden University Medical Center, department of Rheumatology, Leiden, The Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology dpt, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, RappaportFaculty of Medicine - Tehnion, Haifa, Israel. Address correspondence to J.K. de Vries-Bouwstra,; Department of Rheumatology, C01-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands; Email: j.k.de_vries-bouwstra@lumc.nl.
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Marco Matucci-Cerinic
From the Leiden University Medical Center, department of Rheumatology, Leiden, The Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology dpt, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, RappaportFaculty of Medicine - Tehnion, Haifa, Israel. Address correspondence to J.K. de Vries-Bouwstra,; Department of Rheumatology, C01-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands; Email: j.k.de_vries-bouwstra@lumc.nl.
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Alexandra Balbir-Gurman
From the Leiden University Medical Center, department of Rheumatology, Leiden, The Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology dpt, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, V. Pieraccini 18, 50139, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, RappaportFaculty of Medicine - Tehnion, Haifa, Israel. Address correspondence to J.K. de Vries-Bouwstra,; Department of Rheumatology, C01-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, The Netherlands; Email: j.k.de_vries-bouwstra@lumc.nl.
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Abstract

Objective To evaluate agreement of the updated EULAR/EUSTAR recommendations for treatment of systemic sclerosis (SSc) among international experts. To determine factors that might influence agreement.

Methods Level of agreement (10-point scale: 0=not at all, 10=completely agree) and local drug availability (yes/no) were assessed using an online survey. The weblink to the survey was shared with 481 unique email addresses and SSc networks (SCTC, ASIG, INSYNC). Level of agreement was compared between subgroups stratified for participant characteristics.

Results In total 263 experts participated, of whom n=209 (79%) completed each single item. The majority were rheumatologists (n=200, 76%), working in Europe (n=185; 71%); 59% (n=156) were EUSTAR member, and 57% (n = 151) had > 10 years of clinical experience. Overall level of agreement was high (mean 8.0; [SD, 2.5]). Top three highest mean agreement included: 1. ACE-inhibitors for scleroderma renal crisis (9.2[ 2.1]), 2. blood pressure control in SSc-patients treated with corticosteroids (9.0 [2.2]), 3. proton pump inhibitors to prevent reflux complications (9.0[2.2]); top three of lowest mean agreements included: 1. fluoxetine for Raynaud’s phenomenon (RP) (4.6[ 2.8]), 2.hematopoietic stem cell transplantation (HSCT) for severe SSc (7.1[2.9]), 3. phosphodiesterase inhibitors-5 for RP (7.3 [2.7]). Agreement differed between Europe and non-Europe for the use of iloprost, bosentan, methotrexate, HSCT and cyclophosphamide. Treatment availability could partially explain differential agreement for iloprost, bosentan and HSCT.

Conclusion In general, worldwide expert agreement on updated recommendations for treatment of SSc is high, supporting their value. Differences in agreement are partially explained by geographical area and treatment availability.

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Accepted manuscript
Worldwide expert agreement on updated recommendations for the treatment of systemic sclerosis
Jeska de Vries-Bouwstra, Yannick Allanore, Marco Matucci-Cerinic, Alexandra Balbir-Gurman
The Journal of Rheumatology May 2019, jrheum.181173; DOI: 10.3899/jrheum.181173

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Accepted manuscript
Worldwide expert agreement on updated recommendations for the treatment of systemic sclerosis
Jeska de Vries-Bouwstra, Yannick Allanore, Marco Matucci-Cerinic, Alexandra Balbir-Gurman
The Journal of Rheumatology May 2019, jrheum.181173; DOI: 10.3899/jrheum.181173
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