Association of normal nailfold end row loop numbers with a shorter duration of untreated disease in children with juvenile dermatomyositis.

Association of normal nailfold end row loop numbers with a shorter duration of untreated disease in children with juvenile dermatomyositis.
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DOI:
10.1002/art.27379
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发表时间:
2010-05
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:
Ostrowski, Rochella A.;Sullivan, Christine L.;Seshadri, Roopa;Morgan, Gabrielle A.;Pachman, Lauren M.

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确定幼年型皮肌炎(JDM)诊断时的正常终排袢(ERL)与未治疗儿童的临床表现之间的关系,并确定ERL降低的预测因素。收集了80例未经治疗的JDM患儿的临床和实验室资料。在诊断时、24个月和36个月时记录ERL评分。12例ERL正常的儿童与其余68例儿童进行了比较。成果包括:未治疗疾病的持续时间、免疫抑制药物的使用时间、家族病史、疾病活动性评分(DAS)和肌酸磷酸激酶(CPK)、醛缩酶、绝对CD 3 − CD 56 +/16+ NK细胞和血管性血友病因子抗原(vWF:Ag)水平。进行了横向和纵向分析。在诊断时,ERL正常的儿童未治疗疾病的持续时间较短(p=0.03),皮肤DAS较低(p=0.045)。随着时间的推移,异常ERL的可能性增加与未经治疗的疾病持续时间较长和皮肤DAS较高有关。在JDM中,ERL数量正常似乎与症状持续时间较短相关,并且可能是新诊断儿童疾病慢性化的有用指标。正常ERL也与下皮肤DAS相关。正常ERL与其他变量之间缺乏相关性表明,正常NFC不应作为延迟具有典型JDM症状的儿童免疫抑制治疗的理由。
To determine the association of normal end row loops (ERL) at diagnosis of juvenile dermatomyositis (JDM) with clinical findings in untreated children and identify predictors of the development of decreased ERL. Clinical and laboratory data of 80 untreated children with JDM were collected. ERL scores were recorded at time of diagnosis, and at 24 months and 36 months thereafter. Twelve children with normal ERL at diagnosis were compared with the remaining 68 children. Outcomes included: duration of untreated disease, time on immunosuppresive medications, family medical history, disease activity score (DAS), and levels of creatine phosphokinase (CPK), aldolase, absolute CD3−CD56+/16+ NK cells, and von Willebrand factor antigen (vWF:Ag). Cross-sectional and longitudinal analyses were performed. At diagnosis, children with normal ERL had a shorter duration of untreated disease (p=0.03) and a lower skin DAS (p=0.045). Over time, an increased likelihood for having abnormal ERL was associated with a longer duration of untreated disease and with higher skin DAS. The presence of a normal number of ERL in JDM appears to be associated with a shorter duration of symptoms and may be a useful indicator of disease chronicity in the newly diagnosed child. Normal ERL is also associated with lower skin DAS. The lack of association between normal ERL and other variables indicates that normal NFC should not be used as a justification to delay immunosuppressive therapy in children with typical JDM symptoms.
DOI: 10.1093/rheumatology/kel025
发表时间: 2006-08-01
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