Department of Rheumatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Med Sci Monit. 2024 Sep 14;30:e944564. doi: 10.12659/MSM.944564.
Dermatomyositis (DM) is a complex and rare autoimmune disease characterized by muscle weakness and distinctive skin rashes. Its pathogenesis involves a combination of genetic susceptibility, environmental triggers, and immunological factors, with interferon pathways and specific gene upregulations playing crucial roles. Diagnosis is based on clinical presentation, laboratory findings, and imaging, with particular emphasis on myositis-specific antibodies and characteristic muscle and skin changes. The clinical heterogeneity of DM, including variants such as clinically amyopathic DM and DM-associated interstitial lung disease, necessitates a personalized diagnostic and therapeutic approach. Current pharmacological treatments for DM include glucocorticoids, which remain the first-line therapy despite their long-term adverse effects. Immunosuppressants, such as azathioprine, methotrexate, and mycophenolate mofetil, are commonly used in combination with glucocorticoids to enhance efficacy and reduce steroid dependence. Biologics, such as rituximab and intravenous immunoglobulin, have shown effectiveness in refractory cases. Emerging therapies, particularly Janus kinase inhibitors, offer promise for treatment-resistant DM, although they present significant safety concerns, including increased risks of infections and cardiovascular events. Despite significant advancements, managing DM remains challenging due to its rarity and variability. Future research should prioritize the development of precision medicine approaches tailored to individual genetic and pathological features. Additionally, integrated treatment strategies combining pharmacological and non-pharmacological interventions are crucial to improving patient outcomes and quality of life. Understanding the etiology and pathogenesis of DM more deeply will be vital for developing more effective and targeted treatments, ultimately leading to better disease management and prognosis.
皮肌炎(DM)是一种复杂且罕见的自身免疫性疾病,其特征为肌肉无力和独特的皮疹。其发病机制涉及遗传易感性、环境触发因素和免疫因素的综合作用,干扰素途径和特定基因的上调起着关键作用。诊断基于临床表现、实验室发现和影像学检查,特别强调肌炎特异性抗体和特征性肌肉及皮肤改变。DM 的临床异质性,包括临床无肌病型皮肌炎和皮肌炎相关间质性肺病等变体,需要个体化的诊断和治疗方法。目前 DM 的药物治疗包括糖皮质激素,尽管其存在长期不良反应,但仍是一线治疗药物。免疫抑制剂,如硫唑嘌呤、甲氨蝶呤和霉酚酸酯,常与糖皮质激素联合使用,以提高疗效并减少对类固醇的依赖。利妥昔单抗和静脉注射免疫球蛋白等生物制剂在难治性病例中显示出有效性。新兴疗法,特别是 Janus 激酶抑制剂,为治疗抵抗性 DM 提供了希望,但它们存在显著的安全性问题,包括感染和心血管事件风险增加。尽管取得了重大进展,但由于 DM 的罕见性和变异性,其管理仍然具有挑战性。未来的研究应优先考虑开发针对个体遗传和病理特征的精准医学方法。此外,结合药物和非药物干预的综合治疗策略对于改善患者的结局和生活质量至关重要。更深入地了解 DM 的病因和发病机制对于开发更有效和有针对性的治疗方法至关重要,最终将导致更好的疾病管理和预后。