Royal National Hospital for Rheumatic Diseases (Part of the Royal United Hospitals NHS Foundation Trust), Bath, UK.
Department of Pharmacy and Pharmacology, University of Bath, Bath, UK.
Rheumatology (Oxford). 2021 Apr 6;60(4):1593-1607. doi: 10.1093/rheumatology/keaa816.
It is 120 years since 'angiomyositis' was included alongside 'polymyositis' and 'dermatomyositis' in an attempt to propose a taxonomy that reflected the major clinical characteristics of idiopathic inflammatory myopathy (IIM). Endothelial injury, perivascular inflammation and capillary loss are important histological findings in affected tissues in IIM. Overt vascular clinical features including RP and abnormal nailfold capillaroscopy (NC) are also common in IIM. Despite the presence of endothelial injury, perivascular inflammation and capillary loss in affected tissues in IIM, and the presence of clinical features such as RP and NC abnormalities, the pathogenic and therapeutic implications of vasculopathy in IIM have been somewhat overlooked. RP and NC abnormalities are not always present, providing a valuable opportunity to explore aetiopathogenic factors driving vasculopathy within autoimmune rheumatic disease. The present review examines the aetiopathogenic, prognostic and therapeutic significance of vasculopathy in IIM. We describe the prevalence and clinical relevance of vasculopathy in IIM, and consider how vasculopathy may be better utilized to support improved IIM diagnosis and disease classification. Areas of unmet research need are highlighted where relevant.
自 120 年前“血管肌炎”与“多发性肌炎”和“皮肌炎”一起被纳入其中，以试图提出一种反映特发性炎症性肌病（IIM）主要临床特征的分类法以来。内皮损伤、血管周围炎症和毛细血管丧失是 IIM 受累组织的重要组织学发现。在 IIM 中，明显的血管临床特征包括雷诺现象和异常甲襞毛细血管镜（NC）也很常见。尽管 IIM 受累组织存在内皮损伤、血管周围炎症和毛细血管丧失，以及存在 RP 和 NC 异常等临床特征，但血管病变在 IIM 中的发病机制和治疗意义在一定程度上被忽视了。RP 和 NC 异常并不总是存在，这为探索自身免疫性风湿病中血管病变的发病因素提供了宝贵的机会。本综述检查了 IIM 中血管病变的发病机制、预后和治疗意义。我们描述了 IIM 中血管病变的患病率和临床相关性，并考虑了如何更好地利用血管病变来支持改进的 IIM 诊断和疾病分类。在相关情况下，强调了未满足的研究需求领域。
