Khanna Dinesh, Allanore Yannick, Denton Christopher P, Matucci-Cerinic Marco, Pope Janet, Hinzmann Barbara, Davies Siobhan, de Oliveira Pena Janethe, Distler Oliver
Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA.
Rheumatology A Department, Cochin Hospital, Assistance Publique - Hôpitaux de Paris, Paris Descartes University, Paris, France.
J Scleroderma Relat Disord. 2020 Feb;5(1):66-76. doi: 10.1177/2397198319866615. Epub 2019 Aug 21.
Systemic sclerosis is a rare multi-organ autoimmune rheumatic disease, resulting in progressive fibrosis of the skin/internal organs. This study aimed to understand the impact of diffuse cutaneous systemic sclerosis symptoms and disease burden from the patient's perspective.
This was a mixed methodology, market research study involving ethnography, structured interviews, video diaries, and patient tasks. Patients had been diagnosed with diffuse cutaneous systemic sclerosis for ⩾ 6 months and were recruited via healthcare professionals or patient associations (France, Italy, the United Kingdom, and the United States). Patients filmed short (~15 min) daily video diaries about their lives over 7 days and participated in ethnographic sessions, patient tasks, and structured video interviews. In Germany and Spain, patients participated in 60-min telephone interviews.
Twenty-three patients (mean age: 54 years; 83% women; minimum disease duration: 6 months) participated in the study. Time to diagnosis was prolonged, as patients overlooked their symptoms and some healthcare professionals attributed symptoms to other causes. Patients rarely received additional information or support services at diagnosis. Importantly, although patients were aware of the seriousness of organ involvement, they reported that skin changes, pain, and fatigue impaired their ability to perform routine tasks. Patients had a high prescription treatment burden (mean: 10 tablets/day; up to >25 tablets/day) with additional non-prescription medication taken for other comorbidities. Treatment discontinuation was common due to side effects. Patients experienced diffuse cutaneous systemic sclerosis as a loss of independence and self-esteem. Moreover, patients tended to have small support networks, and emotional support services were not offered as standard care.
Patients with diffuse cutaneous systemic sclerosis had high treatment and disease burdens, with skin changes, pain, and fatigue profoundly affecting their lives. There is an unmet need for patient information at the time of diagnosis and emotional support services throughout the patient's journey with diffuse cutaneous systemic sclerosis. Based on the results of this study, we provide recommendations for improving diffuse cutaneous systemic sclerosis care.
系统性硬化症是一种罕见的多器官自身免疫性风湿疾病，会导致皮肤/内脏器官进行性纤维化。本研究旨在从患者角度了解弥漫性皮肤系统性硬化症症状及疾病负担的影响。
这是一项采用混合方法的市场调研研究，涉及人种志、结构化访谈、视频日记和患者任务。患者被诊断为弥漫性皮肤系统性硬化症至少6个月，通过医疗专业人员或患者协会（法国、意大利、英国和美国）招募。患者拍摄了为期7天的关于其生活的简短（约15分钟）每日视频日记，并参加了人种志研究、患者任务和结构化视频访谈。在德国和西班牙，患者参加了60分钟的电话访谈。
23名患者（平均年龄：54岁；83%为女性；最短病程：6个月）参与了研究。诊断时间延长，因为患者忽视自身症状，且一些医疗专业人员将症状归因于其他原因。患者在诊断时很少获得额外信息或支持服务。重要的是，尽管患者意识到器官受累的严重性，但他们报告皮肤变化、疼痛和疲劳损害了他们执行日常任务的能力。患者有很高的处方治疗负担（平均：每天10片；最多>25片/天），还因其他合并症服用额外的非处方药。由于副作用，治疗中断很常见。患者将弥漫性皮肤系统性硬化症体验为独立性和自尊的丧失。此外，患者的支持网络往往较小，情感支持服务并非作为标准护理提供。
弥漫性皮肤系统性硬化症患者有很高的治疗和疾病负担，皮肤变化、疼痛和疲劳对他们的生活产生了深远影响。在诊断时对患者信息以及在弥漫性皮肤系统性硬化症患者整个病程中提供情感支持服务方面存在未满足的需求。基于本研究结果，我们提出了改善弥漫性皮肤系统性硬化症护理的建议。
