Elevated serum interferon-alpha activity in juvenile dermatomyositis: associations with disease activity at diagnosis and after thirty-six months of therapy.

Elevated serum interferon-alpha activity in juvenile dermatomyositis: associations with disease activity at diagnosis and after thirty-six months of therapy.
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DOI:
10.1002/art.24555
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发表时间:
2009-06
影响因子:
--
通讯作者:
Pachman, Lauren M.
Pachman, Lauren M.
中科院分区:
其他
文献类型:
--
作者:
Niewold, Timothy B.;Kariuki, Silvia N.;Morgan, Gabrielle A.;Shrestha, Sheela;Pachman, Lauren M.

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干扰素-α与幼年型皮肌炎的发病机制有关。我们检测了一组幼年糖尿病儿童的血清干扰素-α活性,以确定干扰素-α与疾病活动性和严重性指标之间的关系。39名确诊/可能患有JDM的儿童纳入研究。研究人员对18名新诊断的未经治疗的儿童进行了样本研究,其中11名儿童在接受治疗的24个月时进行了第二次样本采集。其中7名儿童在36个月时也有第三个样本可用,另外21名儿童在首次诊断后36个月进行了研究。用功能报告细胞法测定血清干扰素-α。儿童糖尿病患者血清干扰素-α活性明显高于正常儿童和成人。在未经治疗的患者中,血清干扰素-α活性与血清肌酶呈正相关(P<0.017),与病程呈负相关(P=0.05)。肿瘤坏死因子-α-308a等位基因仅在初治患者中与较高的干扰素-α相关(p=0.038)。在36个月时,在那些仍需要治疗的患者中,血清干扰素-α与肌肉酶呈负相关,在已完成治疗的患者中,与皮肤DAS呈负相关(p=0.002)。在新诊断的患者中,血清干扰素-α活性与较高的血清肌源性酶水平和较短的未治疗病程相关,与诊断后36个月的慢性病活动性指标呈负相关。这些数据提示干扰素-α可能在幼年糖尿病的发病中起作用。
Interferon alpha (IFN-α) has been implicated in the pathogenesis of juvenile dermatomyositis (JDM). We examined serum IFN-α activity in a cohort of children with JDM to determine relationships between IFN-α and indicators of disease activity and severity. 39 children with definite/probable JDM were included in the study. Samples were studied from 18 newly diagnosed untreated children, and 11 of these children had a second sample taken at 24 months while they were receiving treatment. 7 of these children also had a third sample available at 36 months, and 21 additional children were studied 36 months after their initial diagnosis. Serum IFN-α was measured using a functional reporter cell assay. JDM patients had higher serum IFN-α activity than both pediatric and adult healthy controls. In untreated patients, serum IFN-α activity was positively correlated with serum muscle enzymes (p<0.05 for CPK, AST, and aldolase) and inversely correlated with duration of untreated disease (p=0.017). The TNF-α-308A allele was associated with higher serum IFN-α only in untreated patients (p=0.038). At 36 months, serum IFN-α was inversely correlated with muscle enzymes in those patients still requiring therapy, and inversely correlated with skin DAS in those who had completed therapy (p=0.002). Serum IFN-α activity was associated with higher serum levels of muscle derived enzymes and shorter duration of untreated disease in newly diagnosed patients, and inversely correlated with measures of chronic disease activity at 36 months post-diagnosis. These data suggest that IFN-α could play a role in disease initiation in JDM.
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影响因子: 5.5
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