Pellar Russell Edward, Pope Janet Elizabeth
Department of Medicine, University of Western Ontario, London, ON, Canada.
Department of Medicine, University of Western Ontario, London, ON, Canada; St Joseph Health Care, London, ON, Canada.
Semin Arthritis Rheum. 2017 Jun;46(6):767-774. doi: 10.1016/j.semarthrit.2016.12.003. Epub 2016 Dec 9.
Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed.
The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted.
The updated EULAR/EUSTAR guidelines focus specifically on the management of SSc features and include data on newer therapeutic modalities and mention a research agenda. These recommendations are pharmacologic, with few guidelines regarding investigations and non-pharmacologic management. Recommendations from BSR/BHPR are similar to the organ manifestations mentioned in the EULAR/EUSTAR recommendations, and expand on several domains of treatment, including general measures, non-pharmacologic treatment, cardiac involvement, calcinosis, and musculoskeletal features. The guidelines usually agree with one another. Limitations include the lack of guidance for combination or second-line therapy, algorithmic suggestions, the absence of evidence-based recommendations regarding the treatment of specific complications (i.e., gastric antral ectasia and erectile dysfunction). Consensus for when to treat interstitial lung disease in SSc is lacking. There are differences between Europe and North American experts due to access and indications for certain therapies.
Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc and future updates of these recommendations. Care gaps in SSc have been demonstrated so the EULAR/EUSTAR and BSR/BHP guidelines can promote best practices. Certain complications warrant active investigation to further improve outcomes in SSc.
系统性硬化症（SSc）是一种罕见的异质性结缔组织病。需要针对SSc管理中的主要问题提出建议，包括器官并发症的筛查和治疗。
对更新后的欧洲抗风湿病联盟/欧洲硬皮病试验与研究（EULAR/EUSTAR）以及英国风湿病学会（BSR）和英国风湿病健康专业人员（BHPR）指南进行比较和对比。
更新后的EULAR/EUSTAR指南专门关注SSc特征的管理，包括有关新治疗方式的数据，并提及了一项研究议程。这些建议是关于药物治疗的，关于检查和非药物管理的指南很少。BSR/BHPR的建议与EULAR/EUSTAR建议中提到的器官表现相似，并在几个治疗领域进行了扩展，包括一般措施、非药物治疗、心脏受累、钙质沉着和肌肉骨骼特征。这些指南通常相互一致。局限性包括缺乏联合或二线治疗的指导、算法建议，缺乏关于特定并发症（即胃窦扩张和勃起功能障碍）治疗的循证建议。在SSc中何时治疗间质性肺病缺乏共识。由于某些疗法的可及性和适应症，欧洲和北美专家之间存在差异。
已证明SSc存在护理差距，因此EULAR/EUSTAR和BSR/BHP指南可促进最佳实践。某些并发症值得积极研究，以进一步改善SSc的治疗效果和这些建议的未来更新。已证明SSc存在护理差距，因此EULAR/EUSTAR和BSR/BHP指南可促进最佳实践。某些并发症值得积极研究，以进一步改善SSc的治疗效果。
