Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China; Department of Rheumatology, Key Myositis Laboratories, China-Japan Friendship Hospital, Beijing, China.
Department of Rheumatology, Key Myositis Laboratories, China-Japan Friendship Hospital, Beijing, China; Graduate School of Peking Union Medical College, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Semin Arthritis Rheum. 2024 Apr;65:152352. doi: 10.1016/j.semarthrit.2023.152352. Epub 2024 Jan 3.
To depict the clinical panorama of spontaneous pneumomediastinum (SPM) in anti-MDA5 antibody-positive dermatomyositis (anti-MDA5+ DM).
A total of 1352 patients with idiopathic inflammatory myopathy (IIM), including 384 anti-MDA5+ DM patients were retrospectively enrolled. The clinical profiles of anti-MDA5+ DM-associated SPM were analyzed.
We identified that 9.4 % (36/384) of anti-MDA5+ DM patients were complicated with SPM, which was significantly higher than that of non-anti-MDA5+ DM and other IIM subtypes (P all <0.001). SPM developed at a median of 5.5 (3.0, 12.0) months after anti-MDA5+ DM onset. Anti-MDA5+ DM patients complicated with SPM showed a significantly higher frequency of fever, dyspnea, and pulmonary infection including viral and fungal infections compared to those without SPM (P all < 0.05). Cytomegalovirus (CMV) and fungal infections were identified to be independent risk factors for SPM development in the anti-MDA5+ DM. SPM and non-SPM patients in our anti-MDA5+ DM cohort showed comparable short-term and long-term survival (P = 0.236). Furthermore, in the SPM group, we found that the non-survivors had a lower peripheral lymphocyte count, higher LDH level, and higher frequency of intensification of immunosuppressive treatment (IST) than survivors. The elevated LDH level and intensification of IST were independent risk factors for increased mortality in anti-MDA5+ DM-associated SPM patients.
Nearly one-tenth of patients with anti-MDA5+ DM develop SPM. Both CMV and fungal infections are risk factors for SPM occurrence. The development of SPM does not worsen the prognosis of anti-MDA5+ DM patients, and the intensification of IST does harm to the SPM prognosis.
描述抗 MDA5 抗体阳性皮肌炎（anti-MDA5+DM）中自发性纵隔气肿（SPM）的临床概况。
回顾性纳入了 1352 名特发性炎症性肌病（IIM）患者，包括 384 名 anti-MDA5+DM 患者。分析了 anti-MDA5+DM 相关 SPM 的临床特征。
我们发现，9.4%（36/384）的 anti-MDA5+DM 患者并发 SPM，明显高于非 anti-MDA5+DM 和其他 IIM 亚型（均 P<0.001）。SPM 发生在 anti-MDA5+DM 发病后中位 5.5（3.0，12.0）个月。与无 SPM 的患者相比，并发 SPM 的 anti-MDA5+DM 患者发热、呼吸困难和肺部感染（包括病毒和真菌感染）的频率明显更高（均 P<0.05）。巨细胞病毒（CMV）和真菌感染被确定为 anti-MDA5+DM 中 SPM 发生的独立危险因素。在我们的 anti-MDA5+DM 队列中，SPM 和非 SPM 患者的短期和长期生存率无差异（P=0.236）。此外，在 SPM 组中，我们发现非幸存者的外周血淋巴细胞计数较低、LDH 水平较高且强化免疫抑制治疗（IST）的频率较高。LDH 水平升高和 IST 强化是 anti-MDA5+DM 相关 SPM 患者死亡风险增加的独立危险因素。
近十分之一的 anti-MDA5+DM 患者会发生 SPM。CMV 和真菌感染均为 SPM 发生的危险因素。SPM 的发生不会使 anti-MDA5+DM 患者的预后恶化，而 IST 的强化对 SPM 的预后有害。
