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黑色素瘤分化相关基因5阳性皮肌炎中的纵隔气肿:病例系列

Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series.

作者信息

Subramanian Ramaswamy, Prasad Rupal, Mahabaleshwar Mamadapur, Siddiqui Anjum, Ekbote Digvijay, Dhakad Urmila

机构信息

Department of Clinical Immunology and Rheumatology, JSS Medical College and Hospital, JSS Academy of Higher Research, Mysore, India.

Department of Clinical Immunology and Rheumatology, KGMU, Lucknow, India.

出版信息

Mediterr J Rheumatol. 2024 May 21;35(4):660-667. doi: 10.31138/mjr.060124.pim. eCollection 2024 Dec.

Abstract

MDA5+ DM, or anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (DM), is a rare autoimmune illness that primarily affects women of Asian origin. The typical presentation of MDA5+ DM includes a variety of cutaneous lesions accompanied by either no muscular weakness (amyopathic) or hypomyopathic features. In patients with MDA5+ DM, rapid progression of interstitial lung disease is a frequent manifestation associated with poor prognosis. Pneumomediastinum, spontaneous intramuscular haemorrhage, and macrophage activation syndrome form a spectrum of rare manifestations associated with MDA5+ DM. Since these issues are uncommon but fatal, it's important to explore the approaches for diagnosis, treatments, and possible mechanisms, that are useful for prompt treatments and management of the patient.

摘要

黑色素瘤分化相关基因5阳性皮肌炎(MDA5+ DM),或抗黑色素瘤分化相关基因5抗体阳性皮肌炎,是一种罕见的自身免疫性疾病,主要影响亚洲裔女性。MDA5+ DM的典型表现包括各种皮肤病变,伴有无肌肉无力(无肌病型)或低肌病特征。在MDA5+ DM患者中,间质性肺病的快速进展是一种常见表现,与预后不良相关。纵隔气肿、自发性肌肉内出血和巨噬细胞活化综合征构成了与MDA5+ DM相关的一系列罕见表现。由于这些问题不常见但致命,探索诊断、治疗方法及可能的机制很重要,这有助于对患者进行及时治疗和管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b846/11778603/51717065906e/MJR-35-4-660-g001.jpg

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