Disease relapse in idiopathic inflammatory myopathies: A 10-year registry-based follow-up multicentre study in Colombia

Pablo Arango Guerra , Alejandro Echavarría Cross , Manuel Alejandro Giraldo Delgado , Mauricio Murillo Moreno , María Pérez-Restrepo , Juan Camilo Díaz-Coronado , Diego Fernando Rojas-Gualdrón , Neil Pertuz
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Abstract

Introduction

Disease relapses of idiopathic inflammatory myopathies (IIM) play a significant role in the burden of disease. However, Colombian studies with long-term follow-ups are scarce.

Objective

To estimate the ten-year relapse-free survival in patients with idiopathic inflammatory myopathy treated at a specialized medical center for autoimmune diseases with presence in different cities in Colombia.

Method

This is a retrospective follow-up study based on medical records. All patients diagnosed with idiopathic inflammatory myopathy treated between January 2012 and June 2022 were included. The time to relapse was defined as the outcome variable. Additionally, demographic, clinical, laboratory, and treatment variables were characterized. Analyses were performed using Weibull survival regression for interval-censored data.

Results

A total of 278 patients followed for a mean of 6.60 years were included. Of these, 70.9% were women, with a median age of 57 (IQR 40–66) years. Dermatomyositis (60.1%) and polymyositis (28.1%) were the most frequent phenotypes. Relapse-free survival at one year was 86.3%, and at five years, 68.3%, with a median relapse-free time of 13.8 years. By phenotype, the lowest median relapse-free survival rates were observed for antisynthetase syndrome at 3 years, immune-mediated necrotizing myopathy at 6 years, and juvenile dermatomyositis at 7.3 years.

Conclusion

Relapse-free survival is highly heterogeneous according to the clinical phenotype of idiopathic inflammatory myopathy, being lowest in patients with antisynthetase syndrome.
特发性炎性肌病的疾病复发:哥伦比亚一项为期10年的基于登记的随访多中心研究
特发性炎症性肌病(IIM)的疾病复发在疾病负担中起着重要作用。然而,哥伦比亚的长期随访研究很少。目的评估在哥伦比亚不同城市的自身免疫性疾病专科医疗中心治疗的特发性炎性肌病患者的10年无复发生存率。方法基于病历资料进行回顾性随访研究。所有在2012年1月至2022年6月期间接受治疗的特发性炎性肌病患者均被纳入研究。复发时间被定义为结果变量。此外,人口统计学,临床,实验室和治疗变量的特征。使用威布尔生存回归对区间截尾数据进行分析。结果共纳入278例患者,平均随访6.60年。其中70.9%为女性,中位年龄为57岁(IQR 40-66)。皮肌炎(60.1%)和多发性肌炎(28.1%)是最常见的表型。1年无复发生存率为86.3%,5年为68.3%,中位无复发时间为13.8年。根据表型,抗合成酶综合征的中位无复发生存率最低,为3年,免疫介导的坏死性肌病为6年,青少年皮肌炎为7.3年。结论根据特发性炎性肌病的临床表型,患者的无复发生存率存在高度异质性,抗合成酶综合征患者的无复发生存率最低。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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