Kristen L. Chen , Yvonne E. Chiu , Ruth Ann Vleugels , Dominic O. Co , Hanna Kim , Sara E. Sabbagh , Lisa M. Arkin
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引用次数: 0
摘要
幼年皮肌炎(JDM)是最常见的儿科炎症性肌病,尽管治疗手段不断进步,但发病率仍然很高。目前已经出现了不同的临床表型,这些表型与肌炎特异性抗体相关。由于转化数据证实了 I 型 IFNs 在 JDM 疾病发病机制中的作用,因此整合临床和分子表型可能会影响靶向治疗的选择。本文回顾了 JDM 的临床和分子表型以及对免疫发病机制的转化研究,这些研究为靶向治疗提供了新的选择。
Recent Advances in Juvenile Dermatomyositis: Moving toward Integration of Myositis-Specific Antibody Clinical Phenotypes, IFN-Driven Pathogenesis, and Targeted Therapies
Juvenile dermatomyositis (JDM), the most common pediatric inflammatory myopathy, is associated with significant morbidity despite therapeutic advances. Distinct clinical phenotypes have emerged, which can correlate with myositis-specific antibodies. Because translational data solidify the role of type I IFNs in JDM disease pathogenesis, integration of clinical and molecular phenotyping may impact the choice of targeted therapy. This paper reviews clinical and molecular phenotyping in JDM and translational insights into immune pathogenesis that have created emerging options for targeted therapy.
期刊介绍:
Journal of Investigative Dermatology (JID) publishes reports describing original research on all aspects of cutaneous biology and skin disease. Topics include biochemistry, biophysics, carcinogenesis, cell regulation, clinical research, development, embryology, epidemiology and other population-based research, extracellular matrix, genetics, immunology, melanocyte biology, microbiology, molecular and cell biology, pathology, percutaneous absorption, pharmacology, photobiology, physiology, skin structure, and wound healing