Tufts University School of Medicine, Boston, MA, USA.
Department of Dermatology, Tufts Medical Center, Boston, MA, USA.
Int J Dermatol. 2018 Aug;57(8):1007-1014. doi: 10.1111/ijd.14064. Epub 2018 Jun 5.
Discoid lupus erythematosus (DLE) and dermatomyositis (DM) are inflammatory autoimmune diseases that manifest primarily in the skin but can be linked to systemic complications. Although there is an in-depth understanding of the clinical presentation of these two diseases, much less is known regarding the pathophysiology. This has limited the effective treatment options for patients.
An understanding of the pathogenesis of each disease in greater detail will lead to newer targeted medications with less morbidity. This review article endeavors to substantiate the use of new treatments which target the JAK-STAT pathway while elaborating on the immunopathology as well.
PubMed was searched for relevant review articles, case reports, case series reports, randomized clinical trials (RCTs), basic science articles. Appropriate key terms and MeSH terms were utilized in the search. Clinicaltrials.gov was used to find relevant and current clinical trials being conducted in DLE and DM patients.
A review of the literature supports the proposal that though there are likely many components and pathways involved in the destruction of keratinocytes, the Th1 cell immune response and specifically the JAK-STAT signaling pathway is common to both DLE and DM.
Although further study is needed to determine the efficacy and benefits of JAK inhibitors over conventional therapy, these medications should be considered in refractory cases.
盘状红斑狼疮（DLE）和皮肌炎（DM）是主要在皮肤中表现出来的炎症性自身免疫性疾病，但可与全身并发症相关联。尽管人们对这两种疾病的临床表现有深入的了解，但对其病理生理学的了解却要少得多。这限制了患者的有效治疗选择。
更详细地了解每种疾病的发病机制，将导致使用更少发病率的新型靶向药物。本文综述旨在证实使用靶向 JAK-STAT 途径的新疗法的合理性，同时详细阐述免疫病理学。
在 PubMed 上搜索了相关的综述文章、病例报告、病例系列报告、随机临床试验 (RCT)、基础科学文章。在搜索中使用了适当的关键词和 MeSH 术语。Clinicaltrials.gov 用于查找正在进行的针对 DLE 和 DM 患者的相关和当前临床试验。
对文献的回顾支持这样一种观点，即尽管角质形成细胞破坏可能涉及许多成分和途径，但 Th1 细胞免疫反应，特别是 JAK-STAT 信号通路，在 DLE 和 DM 中是共同的。
尽管需要进一步研究来确定 JAK 抑制剂相对于传统疗法的疗效和益处，但在难治性病例中应考虑这些药物。
