Division of Pediatric Rheumatology, Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Cure JM Center of Excellence in Juvenile Dermatomyositis Care and Research, Stanley Manne Children's Research Institute; and The Ann and Robert H. Lurie Children's Hospital of Chicago Research Center, Cure JM Myositis Center, Chicago, Illinois.
J Am Acad Dermatol. 2021 Jun;84(6):1610-1618. doi: 10.1016/j.jaad.2020.12.032. Epub 2020 Dec 25.
Persistent skin manifestations, especially calcinoses, contribute to morbidity in children with juvenile dermatomyositis.
To compare the course of skin and muscle involvement and document frequency of calcinosis in juvenile dermatomyositis.
Prospective cohort study of 184 untreated children with juvenile dermatomyositis (July 1971 to May 2019) at a single children's hospital.
Disease Activity Scores (DASs) were persistently higher for skin versus muscle at all points; clinical inactivity (DAS ≤2) occurred earlier for muscle than skin. Among vascular features for DAS for skin, eyelid margin capillary dilatation was most frequent (54.3%) and persisted longest. Intravenous methylprednisolone reduced DAS for skin more than oral prednisone at 12 months (P = .04). Overall, 16.8% of patients (n = 31) had calcifications, with 4.9% at enrollment. Despite therapy, 25.0% of calcifications recurred and 22.6% failed to resolve; of the latter, 71.4% (n = 5) were present at enrollment. Children with persistent calcifications had longer duration of untreated disease than those whose calcifications resolved (mean 12.5 months) (P < .001). Hydroxychloroquine did not improve DAS for skin (P = .89).
DAS does not quantify nailfold capillary dropout.
In juvenile dermatomyositis, skin disease presents with greater activity and is more recalcitrant to therapies than muscle disease. Early and aggressive treatment can limit the severity and persistence of calcifications identified later in the disease course.
持续性皮肤表现，尤其是钙质沉着症，会增加儿童皮肌炎的发病率。
比较皮肌炎患儿皮肤和肌肉受累的病程，并记录钙质沉着症的发生频率。
对单家儿童医院的 184 例未经治疗的皮肌炎患儿（1971 年 7 月至 2019 年 5 月）进行前瞻性队列研究。
在所有时间点，皮肤疾病活动评分（DAS）均持续高于肌肉 DAS；肌肉的临床无活动（DAS≤2）早于皮肤。在皮肤 DAS 的血管特征中，眼睑缘毛细血管扩张最为常见（54.3%）且持续时间最长。静脉注射甲泼尼龙在 12 个月时比口服泼尼松更能降低皮肤 DAS（P=.04）。总体而言，16.8%的患者（n=31）有钙质沉着，其中 4.9%在入组时存在。尽管进行了治疗，仍有 25.0%的钙质沉着症复发，22.6%的钙质沉着症未得到缓解；其中 71.4%（n=5）在入组时存在。持续性钙质沉着症患儿的未治疗疾病持续时间长于钙质沉着症缓解的患儿（平均 12.5 个月）（P<.001）。羟氯喹并不能改善皮肤 DAS（P=.89）。
DAS 无法量化甲襞毛细血管缺失。
在皮肌炎中，皮肤疾病的活动度更高，且对治疗的反应比肌肉疾病更差。早期和积极的治疗可以限制疾病后期发现的钙质沉着症的严重程度和持续时间。
