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J Rheumatol. 2019 May 1. pii: jrheum.181173. doi: 10.3899/jrheum.181173. [Epub ahead of print]

Worldwide Expert Agreement on Updated Recommendations for the Treatment of Systemic Sclerosis.

Author information

1
From Leiden University Medical Center, Department of Rheumatology, Leiden, the Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology Department, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel. J.K. de Vries-Bouwstra, MD, PhD, Leiden University Medical Center, Department of Rheumatology; Y. Allanore, MD, PhD, Professor, Paris Descartes University, Cochin Hospital, Rheumatology Department; M. Matucci-Cerinic, MD, PhD, Professor, Department of Experimental and Clinical Medicine, University of Florence; A. Balbir-Gurman, MD, PhD, Professor, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine. Address correspondence to Dr. J.K. de Vries-Bouwstra, Department of Rheumatology, C01-R, Leiden University Medical Center, PO Box 9600, 2300 RC Leiden, the Netherlands. E-mail: j.k.de_vries-bouwstra@lumc.nl Accepted for publication April 16, 2019.

Abstract

OBJECTIVE:

To evaluate agreement of the updated European League Against Rheumatism and European Scleroderma Trials and Research group (EUSTAR) recommendations for treatment of systemic sclerosis (SSc) among international experts. In addition, 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 Web link to the survey was shared with 481 unique e-mail addresses and SSc networks (Scleroderma Clinical Trials Consortium, Australian Scleroderma Interest Group, International Systemic Sclerosis Inception Cohort). 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 members; and 57% (n = 151) had > 10 years of clinical experience. Overall level of agreement was high (mean 8.0, SD 2.5). The 3 highest mean agreements included (1) angiotensin-converting enzyme inhibitors for scleroderma renal crisis (9.2, SD 2.1); (2) blood pressure control in SSc-patients treated with corticosteroids (9.0, SD 2.2); (3) proton pump inhibitors to prevent reflux complications (9.0, SD 2.2). The 3 lowest mean agreements included (1) fluoxetine for Raynaud phenomenon (RP; 4.6, SD 2.8); (2) hematopoietic stem cell transplantation (HSCT) for severe SSc (7.1, SD 2.9); (3) phosphodiesterase inhibitors 5 for RP (7.3, SD 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.

PMID:
31043545
DOI:
10.3899/jrheum.181173

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