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

IF 3.2 3区医学 Q2 RHEUMATOLOGY

Rheumatology International Pub Date : 2024-12-01 Epub Date: 2023-07-05 DOI:10.1007/s00296-023-05377-4

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

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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.

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青少年皮肌炎中长期存在且控制不良的疾病与钙质沉着症有关:来自中低收入国家的现实经验。

目的:探讨青少年皮肌炎(JDM)患者钙质沉着的患病率及预测因素。我们回顾了印度北部一家三级风湿病中心20多年的医疗记录，以确定JDM患者，并记录了临床细节。研究了钙质沉着的发生频率、预测因素、特异性治疗及其结果。数据以中位数和四分位数范围表示。在86例JDM患者(中位年龄10岁)中，钙质沉着的发生率为18.2%(首发时为8.5%)。发病年龄较轻、随访时间较长、日球性皮疹[比值比(95%可信区间)11.4(1.4-92.12)]、慢性或多环病程[4.4(1.2-15.5)]和使用环磷酰胺[8.2(1.6-41.9)]与钙沉着症相关。吞咽困难[0.14(0.02-1.2)]和肌肉酶升高[0.14(0.04-0.5)]与钙沉着症呈负相关。用帕米膦酸盐治疗7名儿童中的5名对钙质沉着症有良好到中度的反应。JDM中的钙质沉着症与长期、控制不良的疾病有关，双磷酸盐如帕米膦酸盐的使用为未来的治疗提供了希望。

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来源期刊

Rheumatology International 医学-风湿病学

CiteScore

7.30

自引率

5.00%

发文量

191

审稿时长

16. months

期刊介绍： RHEUMATOLOGY INTERNATIONAL is an independent journal reflecting world-wide progress in the research, diagnosis and treatment of the various rheumatic diseases. It is designed to serve researchers and clinicians in the field of rheumatology. RHEUMATOLOGY INTERNATIONAL will cover all modern trends in clinical research as well as in the management of rheumatic diseases. Special emphasis will be given to public health issues related to rheumatic diseases, applying rheumatology research to clinical practice, epidemiology of rheumatic diseases, diagnostic tests for rheumatic diseases, patient reported outcomes (PROs) in rheumatology and evidence on education of rheumatology. Contributions to these topics will appear in the form of original publications, short communications, editorials, and reviews. "Letters to the editor" will be welcome as an enhancement to discussion. Basic science research, including in vitro or animal studies, is discouraged to submit, as we will only review studies on humans with an epidemological or clinical perspective. Case reports without a proper review of the literatura (Case-based Reviews) will not be published. Every effort will be made to ensure speed of publication while maintaining a high standard of contents and production. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.