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Background <p>Spontaneous pneumomediastinum and/or pneumothorax (SPM/P) have been Linked to unfavorable outcome in anti-melanoma differentiation-associated gene 5 positive dermatomyositis (MDA5<sup>+</sup>DM) patients. This study attempted to investigate the prognostic value of SPM/P and identify the predisposing factors of SPM/P in patients with interstitial lung disease (ILD) complicated with MDA5<sup>+</sup>DM.</p> Methods <p>This study was conducted in a large inception cohort of MDA5<sup>+</sup>DM-ILD (from 2014 to 2023) at Shanghai Renji Hospital. Baseline demographic data, pulmonary function tests, high-resolution CT imaging, laboratory parameters and survival status within 12&#xa0;months were collected and compared between survivors and deceased patients, as well as between patients with and without SPM/P. Kaplan–Meier curves were plotted and Cox regression models were applied to identify prognostic factors. Furthermore, logistic regression analysis was employed to determine the predictors of SPM/P development.</p> Results <p>In a cohort of 523 MDA5<sup>+</sup>DM-ILD patients, 92 (17.6%) developed SPM/P. Of those, 63 SPM/P cases occurred within 3&#xa0;months since ILD onset and had a significantly higher 12-month mortality than those without SPM/P (82.5% <i>versus.</i> 35.4%, <i>p</i> &lt; 0.001). Multivariable Cox regression model identified SPM/P occurred within 3&#xa0;months as an independent risk factor for survival (HR 1.59, 95% CI 1.09–2.34, <i>P</i> = 0.018). Predisposing factors for SPM/P included severe restrictive ventilation dysfunction, male sex, and CMV viremia.</p> Conclusions <p>SPM/P occurred within 3&#xa0;months since ILD onset was identified as a poor prognostic factor in MDA5<sup>+</sup>DM-ILD. A refined FVC%-based staging system combining SPM/P was proposed for better risk stratification and to gauge clinical trial design.</p>

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Spontaneous pneumomediastinum and/or pneumothorax in anti-MDA5 dermatomyositis: a refined staging system

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年10月1日

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  • Jiajia Jin,
  • Wenwen Xu,
  • Huijing Wang,
  • Xiaodong Wang,
  • Qiong Fu,
  • Wanlong Wu,
  • Shuang Ye

摘要

Background

Spontaneous pneumomediastinum and/or pneumothorax (SPM/P) have been Linked to unfavorable outcome in anti-melanoma differentiation-associated gene 5 positive dermatomyositis (MDA5+DM) patients. This study attempted to investigate the prognostic value of SPM/P and identify the predisposing factors of SPM/P in patients with interstitial lung disease (ILD) complicated with MDA5+DM.

Methods

This study was conducted in a large inception cohort of MDA5+DM-ILD (from 2014 to 2023) at Shanghai Renji Hospital. Baseline demographic data, pulmonary function tests, high-resolution CT imaging, laboratory parameters and survival status within 12 months were collected and compared between survivors and deceased patients, as well as between patients with and without SPM/P. Kaplan–Meier curves were plotted and Cox regression models were applied to identify prognostic factors. Furthermore, logistic regression analysis was employed to determine the predictors of SPM/P development.

Results

In a cohort of 523 MDA5+DM-ILD patients, 92 (17.6%) developed SPM/P. Of those, 63 SPM/P cases occurred within 3 months since ILD onset and had a significantly higher 12-month mortality than those without SPM/P (82.5% versus. 35.4%, p < 0.001). Multivariable Cox regression model identified SPM/P occurred within 3 months as an independent risk factor for survival (HR 1.59, 95% CI 1.09–2.34, P = 0.018). Predisposing factors for SPM/P included severe restrictive ventilation dysfunction, male sex, and CMV viremia.

Conclusions

SPM/P occurred within 3 months since ILD onset was identified as a poor prognostic factor in MDA5+DM-ILD. A refined FVC%-based staging system combining SPM/P was proposed for better risk stratification and to gauge clinical trial design.