Department of Rheumatology, Oslo University Hospital, Oslo, Norway.
Department of Rheumatology, University of Zurich, Zurich, Switzerland.
RMD Open. 2019 Mar 4;5(1):e000826. doi: 10.1136/rmdopen-2018-000826. eCollection 2019.
Systemic sclerosis (SSc) is a severe, progressive multiorgan disease but to date, there are no established standardised international guidelines for follow-up of patients with SSc. The goal of this project was to develop an expert consensus for annual systematic investigations in patients with SSc to enhance their standard-of-care.
The Delphi method was applied. All SSc experts from the European Scleroderma Trials and Research group network and the Scleroderma Clinical Trial Consortium were invited to participate. All experts were asked to answer questionnaires in five Delphi steps to determine the domains of interest and tools for each domain for an annual systematic assessment of patients with SSc. Each item was rated on a scale between 0% and 100% (not and very important), and parameters rated >80% by more than 75% of the experts were regarded as acceptable.
In total, 157 experts worldwide participated with 71.3% experts seeing >50 patients with SSc annually. In the first round, 23 domains and 204 tools were suggested. After five Delphi steps, experts agreed on 10 domains including (1) Raynaud's phenomenon; (2) Digital ulcers; (3) Skin and mucosa; (4) Lung; (5); Heart; (6) GI domain, (7) Renal; (8) Musculoskeletal; (9) Laboratory and (10) Treatment. Overall, 55 tools were identified including clinical assessments, laboratory measurements and imaging or functional investigations.
Through five Delphi steps with world leading experts, a consensus was established on strongly suggested tools for a minimum annual systemic assessment of organ involvement in SSc. This work should enhance the standardisation and homogenisation of the practices.
系统性硬化症（SSc）是一种严重的、进行性的多器官疾病，但迄今为止，尚无针对 SSc 患者随访的既定国际标准指南。本项目的目标是制定 SSc 患者年度系统检查的专家共识，以提高其护理标准。
采用德尔菲法。邀请欧洲硬皮病试验和研究组网络和硬皮病临床试验联盟的所有 SSc 专家参与。要求所有专家在五个德尔菲步骤中回答问卷，以确定感兴趣的领域和每个领域的工具，用于对 SSc 患者进行年度系统评估。每个项目的评分范围为 0%至 100%（不重要和非常重要），超过 75%的专家评分>80%的参数被认为是可接受的。
全球共有 157 名专家参与，其中 71.3%的专家每年诊治>50 名 SSc 患者。在第一轮中，提出了 23 个领域和 204 种工具。经过五个德尔菲步骤，专家们就 10 个领域达成一致，包括（1）雷诺现象；（2）指溃疡；（3）皮肤和粘膜；（4）肺；（5）心脏；（6）胃肠道；（7）肾脏；（8）肌肉骨骼；（9）实验室和（10）治疗。总体而言，确定了 55 种工具，包括临床评估、实验室测量以及影像学或功能检查。
通过与世界领先专家进行的五个德尔菲步骤，就 SSc 器官受累的最低年度系统评估强烈建议的工具达成了共识。这项工作应增强实践的标准化和同质化。
