Ostrowski Rochella A, Sullivan Christine L, Seshadri Roopa, Morgan Gabrielle A, Pachman Lauren M
Loyola University Chicago, Chicago, Illinois, USA.
Arthritis Rheum. 2010 May;62(5):1533-8. doi: 10.1002/art.27379.
To determine the association of normal numbers of end row loops (ERLs) in nailfold capillaries at the time of diagnosis of juvenile dermatomyositis (DM) with clinical findings in untreated children with the disease and to identify predictors of the development of decreased numbers of ERLs.
Clinical and laboratory data from 80 untreated children with juvenile DM were collected. ERL numbers were recorded at the time of diagnosis and at 24 months and 36 months thereafter. The 12 children who had normal ERLs at diagnosis were compared with the remaining 68 children. Outcomes included the duration of untreated disease, the duration of treatment with immunosuppressive medications, family medical history, Disease Activity Score (DAS) for juvenile DM, creatinine phosphokinase level, aldolase level, absolute number of CD3-CD56+/16+ natural killer cells, and von Willebrand factor antigen level. Cross-sectional and longitudinal analyses were performed.
At diagnosis, children with normal ERLs had a shorter duration of untreated disease (P = 0.03) and a lower skin DAS (P = 0.045). Over time, an increased likelihood of having decreased numbers of ERLs was associated with a longer duration of untreated disease and with a higher skin DAS.
The presence of a normal number of ERLs in juvenile DM 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 ERLs is also associated with a lower skin DAS. The lack of association between normal ERLs and other variables indicates that normal findings on nailfold capillaroscopy should not be used as justification to delay immunosuppressive therapy in children with typical symptoms of juvenile DM.
确定幼年皮肌炎（DM）诊断时甲襞毛细血管终末排环（ERL）数量正常与未治疗患儿临床特征之间的关联，并确定ERL数量减少的预测因素。
收集80例未治疗的幼年DM患儿的临床和实验室数据。在诊断时、之后24个月和36个月记录ERL数量。将诊断时ERL数量正常的12例患儿与其余68例患儿进行比较。观察指标包括未治疗疾病的持续时间、免疫抑制药物治疗的持续时间、家族病史、幼年DM疾病活动评分（DAS）、肌酸磷酸激酶水平、醛缩酶水平、CD3-CD56+/16+自然杀伤细胞绝对计数以及血管性血友病因子抗原水平。进行横断面和纵向分析。
诊断时，ERL数量正常的患儿未治疗疾病的持续时间较短（P = 0.03），皮肤DAS较低（P = 0.045）。随着时间推移，ERL数量减少的可能性增加与未治疗疾病的持续时间较长和皮肤DAS较高相关。
幼年DM患儿ERL数量正常似乎与症状持续时间较短有关，可能是新诊断患儿疾病慢性化的有用指标。ERL数量正常也与较低的皮肤DAS相关。ERL数量正常与其他变量之间缺乏关联表明，对于有幼年DM典型症状的患儿，甲襞毛细血管镜检查结果正常不应作为延迟免疫抑制治疗的理由。
