Papadopoulou Charalampia, McCann Liza J
Infection, Inflammation, and Rheumatology Section, UCL Great Ormond Street Institute of Child Health, London, United Kingdom.
Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.
Front Pediatr. 2018 Oct 9;6:284. doi: 10.3389/fped.2018.00284. eCollection 2018.
Juvenile dermatomyositis (JDM) is a rare autoimmune disease mainly characterized by muscle and skin involvement. Vasculopathy is considered central to the pathogenesis of the disease. The exact nature of vasculopathy is not yet understood but it is a complex process with both an inflammatory and a non-inflammatory, occlusive component. Impaired function of JDM vasculature includes immune complex deposition, altered expression of cell adhesion molecules predominantly inducing Th17 cell infiltration, and endothelial cell dysfunction. Development of vasculopathy is associated with the severe extra-muscular manifestations of JDM, such as gastrointestinal and cardiac manifestations, interstitial lung disease, ulcerative skin disease or development of calcinosis, and portends a poor prognosis. Correlation of histopathological findings, autoantibodies, and extensive diagnostic workup represent key elements to the early detection of vasculopathic features and early aggressive treatment. Monitoring of vasculopathy remains challenging due to the lack of non-invasive biomarkers. Current treatment approaches provide variable benefit, but better understanding of the essential pathogenic mechanisms should help lead to improved outcomes. Whilst acknowledging that evidence is limited, this review aims to describe the vasculopathy of JDM in the context of pathophysiology, clinical features, and treatment of disease.
幼年皮肌炎（JDM）是一种罕见的自身免疫性疾病，主要特征为肌肉和皮肤受累。血管病变被认为是该疾病发病机制的核心。血管病变的确切性质尚不清楚，但它是一个复杂的过程，既有炎症成分，也有非炎症性的闭塞成分。JDM血管功能受损包括免疫复合物沉积、细胞黏附分子表达改变，主要诱导Th17细胞浸润，以及内皮细胞功能障碍。血管病变的发展与JDM严重的肌肉外表现相关，如胃肠道和心脏表现、间质性肺病、溃疡性皮肤病或钙质沉着的发生，并预示预后不良。组织病理学发现、自身抗体以及广泛诊断检查之间的相关性是早期发现血管病变特征和早期积极治疗的关键要素。由于缺乏非侵入性生物标志物，对血管病变的监测仍然具有挑战性。目前的治疗方法疗效各异，但更好地理解基本致病机制应有助于改善治疗结果。尽管认识到证据有限，但本综述旨在从疾病的病理生理学、临床特征和治疗方面描述JDM的血管病变。
