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青少年皮肌炎中病程长且控制不佳的疾病与钙质沉着有关:来自一个低收入中等收入国家的真实世界经验。

Long-standing and poorly controlled disease in juvenile dermatomyositis is associated with calcinosis: a real-world experience from a low-middle income country.

作者信息

Mehta Pankti, Lawrence Able, Gupta Latika, Misra Durga P, Agarwal Vikas, Misra Ramnath, Aggarwal Amita

机构信息

Department of Clinical Immunology & Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Department of Clinical Immunology & Rheumatology, King George Medical University, Lucknow, India.

出版信息

Rheumatol Int. 2024 Dec;44(12):3035-3040. doi: 10.1007/s00296-023-05377-4. Epub 2023 Jul 5.

Abstract

To study the prevalence and predictors of calcinosis in Juvenile Dermatomyositis (JDM). Medical records over 20 years at a tertiary care rheumatology center in Northern India were reviewed to identify patients with JDM and clinical details were recorded. The frequency of calcinosis, predictors, specific treatment, and its outcomes were studied. Data are expressed as median and interquartile range. In eighty-six patients (median age 10) of JDM, the frequency of calcinosis was 18.2% (8.5% at presentation). Younger age at presentation, longer follow-up, heliotrope rash [Odds Ratio (95% confidence interval), 11.4 (1.4-92.12)], chronic or polycyclic course [4.4 (1.2-15.5)] and cyclophosphamide use [8.2 (1.6-41.9)] were associated with calcinosis. Dysphagia [0.14 (0.02-1.2)] and elevated muscle enzymes [0.14 (0.04-0.5)] were negatively associated with calcinosis. Treatment with pamidronate had a good to moderate response to calcinosis in five of seven children. Calcinosis in JDM is associated with long-standing, poorly controlled disease, and the use of bisphosphonates like pamidronate offer promise in the future for its treatment.

摘要

研究青少年皮肌炎(JDM)中钙质沉着症的患病率及预测因素。回顾了印度北部一家三级医疗风湿病中心20多年的病历,以确定JDM患者并记录临床细节。研究了钙质沉着症的发生率、预测因素、具体治疗方法及其结果。数据以中位数和四分位间距表示。在86例JDM患者(中位年龄10岁)中,钙质沉着症的发生率为18.2%(初诊时为8.5%)。初诊时年龄较小、随访时间较长、向阳疹[比值比(95%置信区间),11.4(1.4 - 92.12)]、慢性或多循环病程[4.4(1.2 - 15.5)]以及使用环磷酰胺[8.2(1.6 - 41.9)]与钙质沉着症相关。吞咽困难[0.14(0.02 - 1.

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