PDF(Pubmed)
Abstract：
Systemic autoinflammatory diseases (SAIDs) are distinct from autoimmune diseases. The former primarily results from abnormal innate immune response and genetic testing is crucial for disease diagnosis. Similar cutaneous involvement is a main feature for both SAID and dermatomyositis (DM), so they can be confused with each other. A literature search of PubMed and MEDLINE was conducted for relevant articles. The similarities and differences between these two types of diseases were analyzed. We found phenotypic similarities between these two types of disorders. Accumulating data supports a major role of the innate immune system and a similar cytokine profile. Molecular testing using an autoinflammatory disease gene panel may help identify SAID patients from the DM population and may offer therapeutic benefit using interleukin-1 (IL-1) inhibitors. A subset of DM, notably amyopathic dermatomyositis in the absence of autoantibodies may be on the spectrum of autoinflammatory disease.
摘要：
系统性自身炎性疾病(SAID)不同于自身免疫疾病。前者主要来自异常的先天免疫反应，基因检测对疾病诊断至关重要。类似的皮肤受累是SAID和皮肌炎（DM）的主要特征，这样他们就会相互混淆.对PubMed和MEDLINE的相关文章进行了文献检索。分析了这两类疾病的异同。我们发现这两种类型的疾病之间的表型相似性。积累的数据支持先天免疫系统的主要作用和相似的细胞因子谱。使用自身炎性疾病基因组的分子测试可能有助于从DM人群中识别SAID患者，并可能使用白介素-1（IL-1）抑制剂提供治疗益处。DM的子集，特别是在缺乏自身抗体的情况下，无肌病性皮肌炎可能是自身炎性疾病的范围。
