Risk factors and disease mechanisms in myositis.
Risk factors and disease mechanisms in myositis.
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DOI：
10.1038/nrrheum.2018.48
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发表时间：
2018-04-20
期刊：
影响因子：
--
通讯作者：
Nagaraju K
中科院分区：
文献类型：
--
作者：
Miller FW;Lamb JA;Schmidt J;Nagaraju K
自身免疫性疾病是由于基因和环境之间的相互作用而引起的慢性炎症。然而，自身免疫性疾病的演变机制仍然知之甚少。特发性炎性肌病（IIM）表型中新发现的危险因素和致病过程为理解这些疾病提供了创新方法。HLA 8.1祖先单倍型是白色人群中主要IIM表型的关键风险因素，其他自身免疫性疾病的遗传风险变体已被确定为IIM风险因素。环境风险因素研究较少，但可能包括病毒，细菌，紫外线辐射，吸烟，职业和围产期暴露以及越来越多的药物，生物制剂和膳食补充剂。疾病机制因表型而异，有证据表明某些表型中存在共享的先天和适应性免疫和代谢途径，但另一些表型中存在独特的途径。IIM的异质性和罕见性使得诊断和治疗的进展变得繁琐。新的方法、更好定义的表型以及国际、多学科的共识都有助于取得进展，希望这些方法最终能够在疾病发作或重大进展之前进行治疗干预。在未来，IIM管理的先发制人的战略可能是可能的。
Autoimmune diseases develop as a result of chronic inflammation owing to interactions between genes and the environment. However, the mechanisms by which autoimmune diseases evolve remain poorly understood. Newly discovered risk factors and pathogenic processes in idiopathic inflammatory myopathy (IIM) phenotypes have illuminated innovative approaches for understanding these diseases. The HLA 8.1 ancestral haplotype is a key risk factor for major IIM phenotypes in white populations, and genetic risk variants for other autoimmune diseases have been identified as IIM risk factors. Environmental risk factors are less studied but might include viruses, bacteria, ultraviolet radiation, smoking, occupational and perinatal exposures and a growing list of drugs, biologics, and dietary supplements. Disease mechanisms vary by phenotype, with evidence for shared innate and adaptive immune and metabolic pathways in some phenotypes but unique pathways in others. The heterogeneity and rarity of the IIMs make advancements in diagnosis and treatment cumbersome. Novel approaches, better-defined phenotypes, and international, multidisciplinary consensus have contributed to progress, and hopefully these methods can eventually enable therapeutic intervention before the onset or major progression of disease. In the future, preemptive strategies to IIM management might be possible.
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