Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.
Royal National Hospital for Rheumatic Diseases (part of Royal United Hospitals), Upper Borough Walls, Bath, UK.
Rheumatology (Oxford). 2019 Jan 1;58(1):18-26. doi: 10.1093/rheumatology/key026.
RP is the most common manifestation of SSc and a major cause of disease-related morbidity. This review provides a detailed appraisal of the patient experience of SSc-RP and potential implications for disease classification, patient-reported outcome instrument development and SSc-RP clinical trial design. The review explores the clinical features of SSc-RP, the severity and burden of SSc-RP symptoms and the impact of SSc-RP on function, work and social participation, body image dissatisfaction and health-related quality of life in SSc. Where management of SSc-RP is concerned, the review focuses on the 'patient experience' of interventions for SSc-RP, examining geographic variation in clinical practice and potential barriers to the adoption of treatment recommendations concerning best-practice management of SSc-RP. Knowledge gaps are highlighted that could form the focus of future research. A more thorough understanding of the patient experience could support the development of novel reported outcome instruments for assessing SSc-RP.
硬皮病相关性间质性肺病(SSc-RP)是硬皮病(SSc)最常见的表现,也是导致疾病相关发病率的主要原因。本文详细评估了 SSc-RP 患者的体验,以及其对疾病分类、患者报告结局(PRO)工具开发和 SSc-RP 临床试验设计的潜在影响。本文探讨了 SSc-RP 的临床特征、SSc-RP 症状的严重程度和负担,以及 SSc-RP 对功能、工作和社会参与、体像不满和 SSc 患者健康相关生活质量的影响。在 SSc-RP 的管理方面,本文重点关注 SSc-RP 干预措施的“患者体验”,检查临床实践中的地域差异,以及在采用 SSc-RP 最佳实践管理的治疗建议方面可能存在的障碍。本文还强调了知识空白,这些空白可能是未来研究的重点。更深入地了解患者的体验可以支持开发用于评估 SSc-RP 的新型报告结局工具。