Abstract：
Dermatomyositis (DM) is a rare inflammatory disease with diverse cutaneous and systemic manifestations, often associated with myositis-specific antibodies. Managing patients with refractory DM, or individuals presenting pecific complications, like calcinosis or rapidly progressive interstitial lung disease, presents unique challenges.
This review explores current and promising treatment options for DM, drawing from clinical studies, case series, and case reports that consider the underlying disease pathophysiology.
Recent advancements have improved our understanding and management of DM. The discovery of distinct DM autoantibodies and their correlation with specific clinical phenotypes has transformed patient categorization and enhanced our knowledge of the pathogenesis of the disease. Intravenous immunoglobulin, a well-established treatment in dermatomyositis, has regained prominence and a large randomized clinical trial has reaffirmed its efficacy, confirming it as an effective therapeutic option in this group of patients. Identification of the type I interferon pathway as a key pathogenic mechanism in DM has opened up new avenues for more effective treatment strategies. Blocking the JAK/STAT pathway offers potential for improved management of refractory patients and prevention of highly morbid complications. These recent advancements have significantly impacted the management and care of dermatomyositis patients, enabling tailored approaches, targeted interventions, and improved outcomes for individuals affected by this complex condition.
This review explores current and promising treatment options for DM, drawing from clinical studies, case series, and case reports that consider the underlying disease pathophysiology.
Recent advancements have improved our understanding and management of DM. The discovery of distinct DM autoantibodies and their correlation with specific clinical phenotypes has transformed patient categorization and enhanced our knowledge of the pathogenesis of the disease. Intravenous immunoglobulin, a well-established treatment in dermatomyositis, has regained prominence and a large randomized clinical trial has reaffirmed its efficacy, confirming it as an effective therapeutic option in this group of patients. Identification of the type I interferon pathway as a key pathogenic mechanism in DM has opened up new avenues for more effective treatment strategies. Blocking the JAK/STAT pathway offers potential for improved management of refractory patients and prevention of highly morbid complications. These recent advancements have significantly impacted the management and care of dermatomyositis patients, enabling tailored approaches, targeted interventions, and improved outcomes for individuals affected by this complex condition.
摘要：
■皮肌炎（DM）是一种罕见的炎症性疾病，具有多种皮肤和全身表现，通常与肌炎特异性抗体有关。管理DM中的皮肤病提出了独特的挑战。
■这篇综述探讨了目前和有希望的DM治疗方案，从临床研究中提取，案例系列,和考虑潜在疾病病理生理学的病例报告。
■最近的进步提高了我们对DM的理解和管理。不同DM自身抗体的发现及其与特定临床表型的相关性改变了患者的分类并增强了我们对疾病发病机理的认识。静脉免疫球蛋白，皮肌炎的一种成熟的治疗方法，重新获得了突出地位，一项大型随机临床试验重申了其疗效，确认它是该组患者的有效治疗选择。I型干扰素途径作为DM的关键致病机制的鉴定为更有效的治疗策略开辟了新的途径。阻断JAK/STAT途径为改善难治性患者的管理和预防高度病态并发症提供了潜力。这些最近的进步显着影响了皮肌炎患者的管理和护理，启用量身定制的方法，有针对性的干预措施，并改善受这种复杂状况影响的个人的结果。
■这篇综述探讨了目前和有希望的DM治疗方案，从临床研究中提取，案例系列,和考虑潜在疾病病理生理学的病例报告。
■最近的进步提高了我们对DM的理解和管理。不同DM自身抗体的发现及其与特定临床表型的相关性改变了患者的分类并增强了我们对疾病发病机理的认识。静脉免疫球蛋白，皮肌炎的一种成熟的治疗方法，重新获得了突出地位，一项大型随机临床试验重申了其疗效，确认它是该组患者的有效治疗选择。I型干扰素途径作为DM的关键致病机制的鉴定为更有效的治疗策略开辟了新的途径。阻断JAK/STAT途径为改善难治性患者的管理和预防高度病态并发症提供了潜力。这些最近的进步显着影响了皮肌炎患者的管理和护理，启用量身定制的方法，有针对性的干预措施，并改善受这种复杂状况影响的个人的结果。
