Pharmacological Strategies in Dermatomyositis: Current Treatments and Future Directions.

IF 3.1 4区 医学 Q1 Medicine Medical Science Monitor Pub Date : 2024-09-14 DOI:10.12659/MSM.944564
Jinqiang Guo, Weiwei Wang, Anbin Huang, Chunli Mei
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Abstract

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.

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皮肌炎的药物治疗策略:皮肌炎的药物治疗策略:当前疗法与未来方向
皮肌炎(Dermatomyositis,DM)是一种复杂而罕见的自身免疫性疾病,以肌肉无力和独特的皮疹为特征。其发病机制涉及遗传易感性、环境诱因和免疫因素的综合作用,其中干扰素通路和特定基因上调起着关键作用。诊断的依据是临床表现、实验室检查结果和影像学检查,重点是肌炎特异性抗体以及特征性肌肉和皮肤变化。由于 DM 的临床异质性,包括临床肌病性 DM 和 DM 相关性间质性肺病等变种,因此有必要采用个性化的诊断和治疗方法。目前对 DM 的药物治疗包括糖皮质激素,尽管它有长期的不良反应,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,通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。生物制剂,如利妥昔单抗和静脉注射免疫球蛋白,已显示出对难治性病例的疗效。新兴疗法,尤其是 Janus 激酶抑制剂,为治疗耐药 DM 带来了希望,不过它们也存在重大的安全隐患,包括增加感染和心血管事件的风险。尽管取得了重大进展,但由于DM的罕见性和多变性,管理DM仍具有挑战性。未来的研究应优先发展针对个体遗传和病理特征的精准医疗方法。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预后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、更有针对性的治疗方法至关重要,最终可改善疾病管理和预后。
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来源期刊
Medical Science Monitor
Medical Science Monitor MEDICINE, RESEARCH & EXPERIMENTAL-
CiteScore
6.40
自引率
3.20%
发文量
514
审稿时长
3.0 months
期刊介绍: Medical Science Monitor (MSM) established in 1995 is an international, peer-reviewed scientific journal which publishes original articles in Clinical Medicine and related disciplines such as Epidemiology and Population Studies, Product Investigations, Development of Laboratory Techniques :: Diagnostics and Medical Technology which enable presentation of research or review works in overlapping areas of medicine and technology such us (but not limited to): medical diagnostics, medical imaging systems, computer simulation of health and disease processes, new medical devices, etc. Reviews and Special Reports - papers may be accepted on the basis that they provide a systematic, critical and up-to-date overview of literature pertaining to research or clinical topics. Meta-analyses are considered as reviews. A special attention will be paid to a teaching value of a review paper. Medical Science Monitor is internationally indexed in Thomson-Reuters Web of Science, Journals Citation Report (JCR), Science Citation Index Expanded (SCI), Index Medicus MEDLINE, PubMed, PMC, EMBASE/Excerpta Medica, Chemical Abstracts CAS and Index Copernicus.
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