Epidemiological characteristics of patients with juvenile dermatomyositis in China: a multicenter study.

Xuanyi Liu,Junmei Zhang,Hui Xu,Weiying Kuang,Jianghong Deng,Xiaohua Tan,Chao Li,Shipeng Li,Yuan Xue,Guoshuang Feng,Caifeng Li,
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Abstract

OBJECTIVE Juvenile dermatomyositis (JDM) is a rare but chronic autoimmune disease with systemic nonsuppurative inflammation. Many studies have focused on the clinical characteristics and therapy of JDM. A few studies have reported the epidemiological characteristics and social burden of JDM patients abroad, but there has been no study in China. METHODS This study was based on the FUTang Updating medical REcords Database. Data was extracted from the registry information of inpatient medical records. The epidemiological characteristics and economic burden of Chinese JDM patients were analyzed. RESULTS A total of 1164 JDM patients from 24 hospitals were enrolled from Jan 2016 to Dec 2021. The ratio of boys to girls was 1:1.23, and half were between 6 and 12 years old. Over half(n=629) were admitted to the hospital at least twice for intensive treatment. 20% of JDM patients suffered from lung involvement and 2.2% suffered from subcutaneous calcification. The median days of hospitalization were 10 (range 6 to 14), in addition, the median United States dollar (USD) of expense was 2370.5(1373.7,3541.9). Lung involvement was the major factor causing high inpatient burden, length of stay, and expense. Nearly, 17% of JDM patients were admitted to the hospital as emergencies, suggesting sever disease activity stage needing urgent treatment. No deaths occurred in our study. CONCLUSION Our study documents the epidemiological characteristics and social burden of JDM patients in China, contributing to the enhanced comprehension and effective management of JDM in the country.
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中国幼年皮肌炎患者的流行病学特征:一项多中心研究。
目的 青少年皮肌炎(JDM)是一种罕见的慢性自身免疫性疾病,伴有全身性非化脓性炎症。许多研究集中于 JDM 的临床特征和治疗。本研究基于 "阜塘更新病历数据库"。数据来自住院病历登记信息。结果从2016年1月至2021年12月,共有来自24家医院的1164名JDM患者入组。男童与女童的比例为1:1.23,半数患者年龄在6至12岁之间。超过一半的患者(n=629)至少两次入院接受强化治疗。20%的JDM患者肺部受累,2.2%的患者皮下钙化。住院天数中位数为 10 天(6-14 天不等),费用中位数为 2370.5 美元(1373.7-3541.9 美元)。肺部受累是造成高住院负担、住院时间和费用的主要因素。近 17% 的 JDM 患者是作为急诊入院的,这表明患者处于需要紧急治疗的严重疾病活动期。结论:我们的研究记录了中国 JDM 患者的流行病学特征和社会负担,有助于提高中国对 JDM 的认识和有效管理。
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