Department of Neurology, Division of Neuromuscular Disease, Brigham and Women's Hospital and Children's Hospital Informatics Program, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.
Ann Rheum Dis. 2011 May;70(5):831-6. doi: 10.1136/ard.2010.139949. Epub 2010 Dec 21.
To determine whether type 1 interferon (IFN) proteins in blood are associated with downstream type 1 IFN-inducible gene expression in blood from patients with myositis.
IFNα, IFNβ and IFNω concentrations were measured by ELISA in 129 blood samples (from 93 patients with dermatomyositis (DM), inclusion body myositis, polymyositis and other muscle diseases and from 36 healthy volunteers). Their concentrations were correlated with their ability to stimulate type 1 IFN-inducible gene transcription in a functional assay for 123 of these samples and the type 1 IFN-inducible blood gene expression from 70 of the same samples.
Blood IFNβ concentration was uniquely associated with DM (p=0.0004), detectable in 64% of samples from patients with untreated or minimally treated DM and 35% of all DM samples compared with 6% of other inflammatory myopathy and 6% of healthy volunteer samples. Blood IFNβ, but not IFNα or IFNω, correlated with high blood type 1 IFN-inducible gene expression (p=0.01). Healthy volunteer samples with a high ELISA signal for IFNα and IFNω lacked functional bioassay activity and such a signal was confirmed as artefactual.
Elevated blood IFNβ protein concentration is associated with DM. Systemic and local production of IFNβ might contribute to, but may not fully explain, the marked overproduction of type 1 IFN-inducible transcripts and proteins seen in DM muscle and blood.
确定血液中的 1 型干扰素 (IFN) 蛋白是否与肌炎患者血液中 1 型 IFN 诱导基因的下游表达相关。
通过 ELISA 法检测 129 份血样（93 份来自皮肌炎 (DM)、包涵体肌炎、多发性肌炎和其他肌肉疾病患者，36 份来自健康志愿者）中的 IFNα、IFNβ 和 IFNω 浓度。将其浓度与 123 份样本的 1 型 IFN 诱导基因转录的功能测定以及 70 份相同样本的 1 型 IFN 诱导血基因表达的能力进行相关分析。
血 IFNβ 浓度与 DM 具有独特的相关性（p=0.0004），在未经治疗或治疗不足的 DM 患者的 64%的样本中可检测到，在所有 DM 样本中占 35%，而在其他炎症性肌病和 6%的健康志愿者样本中占 6%。血 IFNβ 与高血 1 型 IFN 诱导基因表达相关（p=0.01），但 IFNα 或 IFNω 则不然。健康志愿者样本中 IFNα 和 IFNω 的 ELISA 信号高，但缺乏功能生物测定活性，且该信号被确认为人为假象。
血 IFNβ 蛋白浓度升高与 DM 相关。IFNβ 的全身和局部产生可能有助于但不能完全解释 DM 肌肉和血液中 1 型 IFN 诱导转录物和蛋白质的大量过度产生。
