Smith Vanessa, Marjenberg Zoe, Volkmann Elizabeth R
Internal Medicine, Ghent University, Ghent, Belgium; Rheumatology, Ghent University Hospital, Ghent, Belgium; VIB Inflammation Research Center (IRC), Unit for Molecular Immunology and Inflammation, Ghent, Belgium.
Maverex Ltd, Newcastle-upon-Tyne, UK.
Clin Exp Rheumatol. 2025 Aug;43(8):1516-1526. doi: 10.55563/clinexprheumatol/ey5nhr. Epub 2025 Jan 7.
This structured, targeted literature review aimed to assess the mortality, humanistic and economic burden of eight organ manifestations which are commonly experienced by systemic sclerosis patients.
Identification of relevant literature was carried out by searching in Ovid MEDLINE and EMBASE, PubMed, and NHS Economic Evaluation Database in August 2023. Studies reporting original data on patients with systemic sclerosis with at least one of eight organ manifestations (interstitial lung disease and/or pulmonary hypertension, skin, peripheral vascular, musculoskeletal, gastrointestinal, cardiac or renal involvement) published within the last 15 years were included. Meta-analyses with no publication limits were also included.
A total of 50 studies were identified; 37 reported mortality outcomes (including 4 meta-analyses), 9 reported humanistic burden and 11 reported economic burden outcomes. Pulmonary hypertension, cardiac and renal manifestations were generally associated with a poorer survival prognosis. Furthermore, gastrointestinal, skin and peripheral vascular manifestations were found to negatively impact health-related quality of life outcomes. Pulmonary manifestations were associated with substantial economic costs; however, the cost burden of other manifestations is insufficiently reported, despite evidence that they often require healthcare resource use.
Organ manifestations experienced by patients with systemic sclerosis significantly affect patient quality of life and mortality. The economic burden of organ manifestations that are widely experienced by SSc patients such as gastrointestinal issues, is poorly understood and requires further research to quantify and understand. Improvements in diagnosis and clinical management of these systemic sclerosis-associated organ manifestations have the potential for significant alleviation of disease-related burdens.
本项结构化的针对性文献综述旨在评估系统性硬化症患者常见的八种器官表现的死亡率、人文负担和经济负担。
2023年8月，通过检索Ovid MEDLINE、EMBASE、PubMed和英国国家医疗服务体系经济评估数据库来识别相关文献。纳入过去15年内发表的、报告系统性硬化症患者至少有八种器官表现（间质性肺疾病和/或肺动脉高压、皮肤、外周血管、肌肉骨骼、胃肠道, 心脏或肾脏受累）之一的原始数据的研究。也纳入无发表限制的荟萃分析。
共识别出50项研究；37项报告了死亡率结果（包括4项荟萃分析），9项报告了人文负担，11项报告了经济负担结果。肺动脉高压、心脏和肾脏表现通常与较差的生存预后相关。此外，发现胃肠道、皮肤和外周血管表现对健康相关生活质量结果有负面影响。肺部表现与巨大的经济成本相关；然而，尽管有证据表明其他表现常常需要使用医疗资源，但关于它们的成本负担报告不足。
系统性硬化症患者经历的器官表现显著影响患者的生活质量和死亡率。系统性硬化症患者广泛经历的器官表现（如胃肠道问题）的经济负担了解不足，需要进一步研究以量化和理解。改善这些与系统性硬化症相关的器官表现的诊断和临床管理有可能显著减轻疾病相关负担。
