Jung Junghee, Kim Min Jee, Yoo Bin, Lee Chang-Keun, Kim Yong-Gil, Hong Seokchan, Ahn Soo Min, Kim Ho Cheol
Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
PLoS One. 2025 Jul 11;20(7):e0328043. doi: 10.1371/journal.pone.0328043. eCollection 2025.
Idiopathic inflammatory myositis (IIM) frequently coexists with interstitial lung disease (ILD), significantly impacting morbidity and mortality. Spontaneous pneumomediastinum, a complication of myositis-associated ILD, remains understudied regarding its clinical implications.
We retrospectively reviewed patients diagnosed with myositis-associated ILD at Asan Medical Center, Seoul, South Korea, from April 2012 to September 2023. Patients were categorized into two groups based on the presence or absence of spontaneous pneumomediastinum during the follow-up period.
Among the 70 patients included in the study, the median age was 55.9 ± 12.2 years, with 62.9% being female. Pneumomediastinum developed in 12 (17.1%) patients. Clinical characteristics did not significantly differ between the pneumomediastinum and non-pneumomediastinum groups, except for the subtype of IIM. Notably, pneumomediastinum was observed in 11 (91.7%) patients with dermatomyositis and 1 (8.3%) with anti-synthetase syndrome (ASS), but none with polymyositis. Multivariate analysis revealed pneumomediastinum as a significant risk factor for mortality (hazard ratio: 2.829, 95% confidence interval: 1.100-7.270, p = 0.031) after adjusting for other variables. Patients with pneumomediastinum exhibited worse survival compared with patients without pneumomediastinum (median survival time: 77.7 ± 11.7 vs. 13.6 ± 3.7 months, p = 0.013).
Spontaneous pneumomediastinum is an independent risk factor for mortality in patients with myositis-associated ILD.
特发性炎症性肌病（IIM）常与间质性肺疾病（ILD）共存，对发病率和死亡率有显著影响。自发性纵隔气肿作为肌炎相关ILD的一种并发症，其临床意义仍未得到充分研究。
我们回顾性分析了2012年4月至2023年9月在韩国首尔峨山医疗中心诊断为肌炎相关ILD的患者。根据随访期间是否存在自发性纵隔气肿将患者分为两组。
在纳入研究的70例患者中，中位年龄为55.9±12.2岁，女性占62.9%。12例（17.1%）患者发生了纵隔气肿。除IIM亚型外，纵隔气肿组和非纵隔气肿组的临床特征无显著差异。值得注意的是，11例（91.7%）皮肌炎患者和1例（8.3%）抗合成酶综合征（ASS）患者出现了纵隔气肿，但多发性肌炎患者均未出现。多变量分析显示，在调整其他变量后，纵隔气肿是死亡率的一个显著危险因素（风险比：2.829，95%置信区间：1.100-7.270，p=0.031）。与无纵隔气肿的患者相比，有纵隔气肿的患者生存率更差（中位生存时间：77.7±11.7个月 vs. 13.6±3.7个月，p=0.013）。
自发性纵隔气肿是肌炎相关ILD患者死亡的独立危险因素。
