Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA.
Division of Rheumatology, Rush University Medical Center, Chicago, IL, USA.
Autoimmun Rev. 2020 Jun;19(6):102533. doi: 10.1016/j.autrev.2020.102533. Epub 2020 Mar 28.
Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by muscle weakness and cutaneous manifestations in adults and children. Calcinosis, a complication of DM, is the abnormal deposition of insoluble calcium salts in tissues, including skin, subcutaneous tissue, tendons, fascia, and muscle. Calcinosis is more commonly seen in juvenile DM (JDM), but also develops in adult DM. Although the mechanism of calcinosis remains unclear, several pathogenic hypotheses have been proposed, including intracellular accumulation of calcium secondary to an alteration of the cellular membrane by trauma and inflammation, local vascular ischemia, dysregulation of mechanisms controlling the deposition and solubility of calcium and phosphate, and mitochondrial damage of muscle cells. Identifying calcinosis biomarkers is important for early disease detection and risk assessment, and may lead to novel therapeutic targets for the prevention and treatment of DM-associated calcinosis. In this review, we summarize myositis autoantibodies associated with calcinosis in DM, histopathology and chemical composition of calcinosis, genetic and inflammatory markers that have been studied in adult DM and JDM-associated calcinosis, as well as potential novel biomarkers.
皮肌炎(DM)是一种罕见的特发性炎症性肌病,以成人和儿童的肌肉无力和皮肤表现为特征。钙沉积症是 DM 的一种并发症,是不溶性钙盐在组织中的异常沉积,包括皮肤、皮下组织、肌腱、筋膜和肌肉。钙沉积症在幼年型皮肌炎(JDM)中更为常见,但也会在成年型皮肌炎中发生。尽管钙沉积症的发病机制仍不清楚,但已经提出了几种发病假说,包括创伤和炎症引起的细胞膜改变导致细胞内钙积累、局部血管缺血、控制钙和磷酸盐沉积和解离的机制失调以及肌肉细胞的线粒体损伤。识别钙沉积症的生物标志物对于早期疾病检测和风险评估很重要,并且可能为预防和治疗与 DM 相关的钙沉积症提供新的治疗靶点。在这篇综述中,我们总结了与 DM 中的钙沉积症相关的肌炎自身抗体、钙沉积症的组织病理学和化学成分、在成人 DM 和 JDM 相关钙沉积症中研究过的遗传和炎症标志物,以及潜在的新型生物标志物。