University of Colorado School of Medicine, Denver, CO, USA.
RTI Health Solutions, Manchester, United Kingdom.
Autoimmun Rev. 2017 Nov;16(11):1147-1154. doi: 10.1016/j.autrev.2017.09.010. Epub 2017 Sep 9.
Systemic sclerosis (SSc), or systemic scleroderma, is a chronic multisystem autoimmune disease characterised by widespread vascular injury and progressive fibrosis of the skin and internal organs. Patients with SSc have decreased survival, with pulmonary involvement as the main cause of death. Current treatments for SSc manage a range of symptoms but not the cause of the disease. Our review describes the humanistic and cost burden of SSc.
A structured review of the literature was conducted, using predefined search strategies to search PubMed, Embase, and the Cochrane Library. Grey literature searches also were conducted.
In total, 2226 articles were identified in the databases and 52 were included; an additional 10 sources were included from the grey literature. The review identified six studies reporting relevant cost estimates conducted in five different countries and four studies that assessed the humanistic burden of SSc. Total direct annual medical costs per patient for Europe varied from €3544 to €8452. For Canada, these costs were reported to be from Can$5038 to Can$10,673. In the United States, the total direct health care costs were reported to be US$17,365 to US$18,396. Different key drivers of direct costs were reported, including hospitalisations, outpatients, and medication. The total annual costs per patient were reported at Can$18,453 in Canada and varied from €11,074 to €22,459 in Europe. Indirect costs represented the largest component of the total costs. EQ-5D utility scores were lower for patients with SSc than those observed in the general population, with reported mean values of 0.49 and 0.68, respectively. The average value of the Health Assessment Questionnaire for patients with SSc was significantly higher than the control population (0.94), and the average value of the SF-36 was significantly lower than the control population: 49.99 for the physical dimension and 58.42 for the mental dimension.
Overall, there is a paucity of information on the burden of SSc. Nonetheless, our review indicates that the quality of life of patients with SSc is considerably lower than that of the general population. In addition, SSc places a considerable economic burden on health care systems and society as a whole.
系统性硬化症（SSc），又称系统性硬皮病，是一种慢性多系统自身免疫性疾病，其特征为广泛的血管损伤和皮肤及内脏器官的进行性纤维化。SSc 患者的生存率降低，肺部受累是主要死亡原因。目前 SSc 的治疗方法可缓解多种症状，但不能针对病因进行治疗。本综述描述了 SSc 的人文和经济负担。
使用预先设定的搜索策略，对 PubMed、Embase 和 Cochrane 图书馆进行了系统文献检索，并进行了灰色文献检索，以进行文献综述。
数据库中共检索到 2226 篇文章，纳入 52 篇，从灰色文献中纳入 10 篇。综述共识别出 6 项在五个不同国家开展的与相关费用估计相关的研究，以及 4 项评估 SSc 人文负担的研究。欧洲每位患者每年的直接医疗总费用从 3544 欧元到 8452 欧元不等。加拿大的这些费用报告为 5038 加元到 10673 加元。在美国，总直接医疗保健费用报告为 17365 美元到 18396 美元。不同的直接费用主要驱动因素包括住院、门诊和药物治疗。在加拿大，每位患者每年的总成本报告为 18453 加元，欧洲的总成本报告为 11074 欧元到 22459 欧元不等。间接成本是总费用的最大组成部分。SSc 患者的 EQ-5D 效用评分低于普通人群，分别为 0.49 和 0.68。SSc 患者健康评估问卷的平均值明显高于对照组（0.94），而 SF-36 的平均值明显低于对照组：身体维度为 49.99，心理维度为 58.42。
总体而言，SSc 负担的相关信息较少。尽管如此，我们的综述表明 SSc 患者的生活质量明显低于普通人群。此外，SSc 给医疗保健系统和整个社会带来了相当大的经济负担。
