Systemic sclerosis: state of the art on clinical practice guidelines.
Systemic sclerosis: state of the art on clinical practice guidelines.
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DOI：
10.1136/rmdopen-2018-000782
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发表时间：
2018
期刊：
影响因子：
6.2
通讯作者：
Matucci-Cerinic M
中科院分区：
文献类型：
--
作者：
Smith V;Scirè CA;Talarico R;Airo P;Alexander T;Allanore Y;Bruni C;Codullo V;Dalm V;De Vries-Bouwstra J;Della Rossa A;Distler O;Galetti I;Launay D;Lepri G;Mathian A;Mouthon L;Ruaro B;Sulli A;Tincani A;Vandecasteele E;Vanhaecke A;Vanthuyne M;Van den Hoogen F;Van Vollenhoven R;Voskuyl AE;Zanatta E;Bombardieri S;Burmester G;Eurico FJ;Frank C;Hachulla E;Houssiau F;Mueller-Ladner U;Schneider M;van Laar JM;Vieira A;Cutolo M;Mosca M;Matucci-Cerinic M
系统性硬化症（SSc）是一种罕见疾病，其特征是自身免疫、皮肤和内脏纤维化以及血管病变。SSc可能与高发病率和死亡率相关。在这篇叙述性综述中，我们总结了一项系统性文献研究的结果，该研究是欧洲罕见和复杂结缔组织和肌肉骨骼疾病参考网络项目的一部分，旨在评估现有的临床实践指南或建议。只有在“血管和溃疡”（即，指端溃疡的非药物方法）、“PAH”（即，筛查和治疗）、“治疗”和“青少年”（即，雷诺现象青少年的评估）领域，才能纳入循证和共识指南。因此，在SSc中，涉及几种SSc特异性并发症的诊断和（非）药物治疗的未满足需求占优势。SSc患者在SSc相关分类、管理（药物和非药物）和教育方面存在显著的不确定性。这种疾病的日常影响（丧失自尊、疲劳、性功能障碍以及职业、营养和关系问题）被低估，需要进行评估。
Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains ‘Vascular & Ulcers’ (ie, non-pharmacological approach to digital ulcer), ‘PAH’ (ie, screening and treatment), ‘Treatment’ and ‘Juveniles’ (ie, evaluation of juveniles with Raynaud’s phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.
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