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抗黑色素瘤分化相关基因5(抗MDA5)皮肌炎相关间质性肺疾病并发纵隔气肿:一例报告及文献综述

Anti-melanoma Differentiation-Associated Gene 5 (Anti-MDA5) Dermatomyositis-Associated Interstitial Lung Disease Complicated by Pneumomediastinum: A Case Report and Literature Review.

作者信息

Chaudhary Shivang, Balagani Amulya, Zaheer Muhammad

机构信息

Internal Medicine, Saint Louis University School of Medicine, St. Louis, USA.

Rheumatology, Saint Louis University School of Medicine, St. Louis, USA.

出版信息

Cureus. 2024 Jul 9;16(7):e64191. doi: 10.7759/cureus.64191. eCollection 2024 Jul.

Abstract

Anti-melanoma differentiation-associated gene 5 (anti-MDA5) dermatomyositis (DM) is a subset of amyopathic myositis and is associated with unique cutaneous manifestations and rapidly progressive interstitial lung disease (RP-ILD). A rare complication associated with high mortality is the occurrence of pneumomediastinum. We present a case of a 58-year-old female with anti-MDA5 DM-associated interstitial lung disease (ILD) complicated by pneumomediastinum. Treatment with pulse dose steroids and intravenous cyclophosphamide led to clinical improvement and resolution of the pneumomediastinum. Our case emphasizes the recognition of ILD-associated pneumomediastinum in patients with anti-MDA5 DM. Swift diagnosis and aggressive treatment are crucial due to the associated high mortality.

摘要

抗黑色素瘤分化相关基因5(抗MDA5)皮肌炎(DM)是无肌病性肌炎的一个亚型,与独特的皮肤表现和快速进展性间质性肺病(RP-ILD)相关。纵隔气肿是一种罕见且死亡率高的并发症。我们报告一例58岁女性,患有抗MDA5 DM相关间质性肺病(ILD)并伴有纵隔气肿。脉冲剂量类固醇和静脉注射环磷酰胺治疗使临床症状改善,纵隔气肿消退。我们的病例强调了对抗MDA5 DM患者ILD相关纵隔气肿的认识。由于相关死亡率高,快速诊断和积极治疗至关重要。

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