PDF(Pubmed)
Abstract：
UNASSIGNED: Autoantibodies to type I interferons have been identified in association with a variety of inflammatory and autoimmune diseases. Type I interferons have demonstrated inhibitory effects on mast cell proliferation and degranulation. Systemic mastocytosis (SM) is a disease characterized by increased mast cell burden and mediator release. Whether autoantibodies to type I interferon are present in the sera of patients with SM, and if so, whether they correlate with characteristics of disease, is unknown.
UNASSIGNED: The purpose of this study was to determine whether autoantibodies to type I interferons are observed in the sera of patients with SM, and if so, whether they correlate with biomarkers of disease severity.
UNASSIGNED: We analyzed sera from 89 patients with SM for concentrations of autoantibodies to type I interferon by using a multiplex particle-based assay and signal neutralization capacity by using a STAT1 activity assay and then compared these measurements with those in a database of information on 1284 healthy controls.
UNASSIGNED: Our cohort was predominantly female (57.3%), with a median age of 56 years. Of the cohort members, 13 produced autoantibodies to IFN-β, 3 to IFN-ω, and 0 to IFN-α. None of the 13 sera demonstrated signal neutralization. Neither autoantibody concentration nor signaling inhibition measurements correlated with tryptase concentrations or D816V allele burden.
UNASSIGNED: Although a small subpopulation of patients with SM have autoantibodies to type I interferons, there was no correlation between autoantibody production and signaling inhibition. These data are consistent with the conclusion that autoantibodies to type I interferon do not play a significant role in the pathogenesis or severity of SM.
UNASSIGNED: The purpose of this study was to determine whether autoantibodies to type I interferons are observed in the sera of patients with SM, and if so, whether they correlate with biomarkers of disease severity.
UNASSIGNED: We analyzed sera from 89 patients with SM for concentrations of autoantibodies to type I interferon by using a multiplex particle-based assay and signal neutralization capacity by using a STAT1 activity assay and then compared these measurements with those in a database of information on 1284 healthy controls.
UNASSIGNED: Our cohort was predominantly female (57.3%), with a median age of 56 years. Of the cohort members, 13 produced autoantibodies to IFN-β, 3 to IFN-ω, and 0 to IFN-α. None of the 13 sera demonstrated signal neutralization. Neither autoantibody concentration nor signaling inhibition measurements correlated with tryptase concentrations or D816V allele burden.
UNASSIGNED: Although a small subpopulation of patients with SM have autoantibodies to type I interferons, there was no correlation between autoantibody production and signaling inhibition. These data are consistent with the conclusion that autoantibodies to type I interferon do not play a significant role in the pathogenesis or severity of SM.
摘要：
■已经鉴定了I型干扰素的自身抗体与多种炎症和自身免疫疾病相关。I型干扰素已证明对肥大细胞增殖和脱颗粒的抑制作用。系统性肥大细胞增多症（SM）是一种以肥大细胞负荷和介质释放增加为特征的疾病。SM患者的血清中是否存在针对I型干扰素的自身抗体，如果是这样,它们是否与疾病的特征相关，是未知的。
■这项研究的目的是确定在SM患者的血清中是否观察到针对I型干扰素的自身抗体，如果是这样,它们是否与疾病严重程度的生物标志物相关。
■我们通过使用基于多重颗粒的测定和使用STAT1活性测定的信号中和能力，分析了89名SM患者的血清中针对I型干扰素的自身抗体浓度，然后将这些测量值与1284名健康对照信息数据库中的测量值进行了比较。
■我们的队列主要是女性（57.3%），平均年龄为56岁。在队列成员中，13产生了针对IFN-β的自身抗体，3到IFN-ω，和0到IFN-α。13份血清均未显示信号中和。自身抗体浓度和信号传导抑制测量均与类胰蛋白酶浓度或D816V等位基因负荷无关。
■尽管一小部分SM患者具有I型干扰素的自身抗体，自身抗体产生和信号抑制之间没有相关性.这些数据与I型干扰素的自身抗体在SM的发病机理或严重程度中不发挥重要作用的结论一致。
■这项研究的目的是确定在SM患者的血清中是否观察到针对I型干扰素的自身抗体，如果是这样,它们是否与疾病严重程度的生物标志物相关。
■我们通过使用基于多重颗粒的测定和使用STAT1活性测定的信号中和能力，分析了89名SM患者的血清中针对I型干扰素的自身抗体浓度，然后将这些测量值与1284名健康对照信息数据库中的测量值进行了比较。
■我们的队列主要是女性（57.3%），平均年龄为56岁。在队列成员中，13产生了针对IFN-β的自身抗体，3到IFN-ω，和0到IFN-α。13份血清均未显示信号中和。自身抗体浓度和信号传导抑制测量均与类胰蛋白酶浓度或D816V等位基因负荷无关。
■尽管一小部分SM患者具有I型干扰素的自身抗体，自身抗体产生和信号抑制之间没有相关性.这些数据与I型干扰素的自身抗体在SM的发病机理或严重程度中不发挥重要作用的结论一致。
