Department of Rheumatology, Cairo University Hospitals, Cairo University, Egypt.
Department of Paediatric, Cairo University Hospitals, Cairo University, Egypt.
Clin Exp Rheumatol. 2022 Feb;40(2):450-456. doi: 10.55563/clinexprheumatol/h0s7tq. Epub 2021 Jul 21.
To study the demographics, characteristics, management and disease outcome of Egyptian children with juvenile dermatomyositis (JDM).
Retrospective analysis of the records of 134 JDM patients attending two centres in Cairo, Egypt from January 2010 to December 2019. A total of 128 patients were included in the study, all of which fulfilled either the Bohan and Peter criteria and/or the EULAR/ACR classification criteria of 2017.
The mean age of disease onset was 5.9±2.8 years and the follow-up duration were 6±3.2 years. Female to male ratio was 2.2:1. Constitutional manifestations and cutaneous skin ulcers were common, while gut vasculopathy was rare in our patients. Heliotrope rash was the commonest skin manifestation. Lactate dehydrogenase enzyme was more frequently elevated than creatine kinase. Electromyography was the most frequently used diagnostic procedure, while muscle biopsy and muscle MRI were not commonly done in our patients. Glucocorticoids, methotrexate, hydroxychloroquine, mycophenolate mofetil and IVIG were the most frequently used medications. Sixty (46.9 %) of the patients had clinically inactive disease, at the last follow-up visit. Chronic skin disease, residual muscle weakness, calcinosis and growth failure were among the most common cumulative damage manifestations. The mortality rate was 1.6% over the follow-up period, one death was due to severe infection, and the other due to respiratory failure.
Although our patients shared several similarities with their peers in the Middle East and in Europe, there were some striking differences. These differences can be attributed to the ethnic and environmental disparities.
研究埃及儿童幼年特发性皮肌炎（JDM）的人口统计学、特征、治疗方法和疾病结局。
回顾性分析 2010 年 1 月至 2019 年 12 月在埃及开罗的两个中心就诊的 134 例 JDM 患者的记录。共有 128 例患者纳入本研究，均符合 Bohan 和 Peter 标准和/或 2017 年 EULAR/ACR 分类标准。
疾病发病的平均年龄为 5.9±2.8 岁，随访时间为 6±3.2 年。女性与男性比例为 2.2:1. 常见的全身症状和皮肤溃疡，而肠道血管病变在我们的患者中很少见。眶周皮疹是最常见的皮肤表现。乳酸脱氢酶酶比肌酸激酶更常升高。肌电图是最常用的诊断程序，而肌肉活检和肌肉 MRI 在我们的患者中并不常见。糖皮质激素、甲氨蝶呤、羟氯喹、霉酚酸酯和 IVIG 是最常用的药物。在最后一次随访时，60（46.9%）例患者疾病处于临床缓解期。慢性皮肤疾病、残留肌肉无力、钙质沉着和生长发育不良是最常见的累积损害表现。在随访期间死亡率为 1.6%，1 例死亡是由于严重感染，另 1 例是由于呼吸衰竭。
尽管我们的患者与中东和欧洲的同龄人有一些相似之处，但也存在一些明显的差异。这些差异可以归因于种族和环境差异。
