Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis
Persistent association of nailfold capillaroscopy changes and skin involvement over thirty-six months with duration of untreated disease in patients with juvenile dermatomyositis
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DOI：
10.1002/art.23299
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发表时间：
2008-02-01
影响因子：
--
通讯作者：
Pachman, Lauren M.
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文献类型：
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作者：
Christen-Zaech, Stephanie;Seshadri, Roopa;Pachman, Lauren M.
客观的。确定甲襞毛细管镜检查变化与幼年皮肌炎 (DM) 儿童临床表现和基因型的关联，以确定 36 个月内病程的潜在差异。方法。在开始治疗之前，在 61 名儿童诊断为青少年 DM 时，测定肿瘤坏死因子 α (TNF α) -308 等位基因和 DQA1*0501 状态，获得青少年 DM 疾病活动评分 (DAS)，并进行甲襞毛细血管镜检查。监测病程36个月。通过回归分析评估患者内部和患者之间的变化。结果。诊断时，未治疗疾病持续时间较短 (P = 0.05) 和青少年 DM 皮肤 DAS 较低 (P = 0.035) 与单周期病程相关。 36 个月内，行尾环 (ERL) 再生与下层皮肤 DAS 相关（P < 0.001），但与肌肉 DAS 无关（P = 0.98）； ERL 再生和浓密环减少与未治疗疾病持续时间较短相关（两者 P = 0.04）。 36 个月时，ERL 再生增加 (P = 0.007) 以及皮肤 DAS (P < 0.001) 和肌肉 DAS (P = 0.025) 的改善与单周期病程相关。结论。青少年糖尿病的早期治疗可能导致单循环病程。非单周期病程通常涉及持续的皮肤表现和持续的甲襞毛细血管镜检查变化。 36 个月内，甲襞毛细血管镜检查结果与青少年 DM 皮肤症状而非肌肉骨骼症状的相关性表明，皮肤和肌肉血管病变具有不同的病理生理机制。这些发现表明，需要更多地关注确定青少年 DM 皮肤特征的最佳治疗方法。
Objective. To determine the association of changes on nailfold capillaroscopy with clinical findings and genotype in children with juvenile dermatomyositis (DM), in order to identify potential differences in disease course over 36 months.Methods. At diagnosis of juvenile DM in 61 children prior to the initiation of treatment, tumor necrosis factor alpha (TNF alpha) -308 allele and DQA1*0501 status was determined, juvenile DM Disease Activity Scores (DAS) were obtained, and nailfold capillaroscopy was performed. The disease course was monitored for 36 months. Variations within and between patients were assessed by regression analysis.Results. At diagnosis, shorter duration of untreated disease (P = 0.05) and a lower juvenile DM skin DAS (P = 0.035) were associated with a unicyclic disease course. Over 36 months, end-row loop (ERL) regeneration was associated with lower skin DAS (P < 0.001) but not muscle DAS (P = 0.98); ERL regeneration and decreased bushy loops were associated with a shorter duration of untreated disease (P = 0.04 for both). At 36 months, increased ERL regeneration (P = 0.007) and improvement of skin DAS (P < 0.001) and muscle DAS (P = 0.025) were associated with a unicyclic disease course.Conclusion. Early treatment of juvenile DM may lead to a unicyclic disease course. The non-unicyclic disease course usually involves continuing skin manifestations with persistent nailfold capillaroscopy changes. The correlation of nailfold capillaroscopy results with cutaneous but not with musculoskeletal signs of juvenile DM over a 36-month period suggests that the cutaneous and muscle vasculopathies have different pathophysiologic mechanisms. These findings indicate that efforts to identify the optimal treatment of cutaneous features in juvenile DM require greater attention.