{"title":"Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series.","authors":"Ramaswamy Subramanian, Rupal Prasad, Mamadapur Mahabaleshwar, Anjum Siddiqui, Digvijay Ekbote, Urmila Dhakad","doi":"10.31138/mjr.060124.pim","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":32816,"journal":{"name":"Mediterranean Journal of Rheumatology","volume":"35 4","pages":"660-667"},"PeriodicalIF":0.0000,"publicationDate":"2024-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11778603/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Mediterranean Journal of Rheumatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.31138/mjr.060124.pim","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/12/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
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.