Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry

被引:76
作者
Mehta, Pankti [1 ]
Machado, Pedro M. [2 ,3 ,4 ,5 ]
Gupta, Latika [1 ]
机构
[1] Sanjay Gandhi Post Grad Inst Med Sci SGPGIMS, Dept Clin Immunol & Rheumatol, Rae Bareilly Rd, Lucknow 226014, Uttar Pradesh, India
[2] Univ Coll London Hosp NHS Fdn Trust, Univ Coll London Hosp, Biomed Res Ctr, Natl Inst Hlth Res NIHR, London, England
[3] London North West Univ Healthcare NHS Trust, Northwick Pk Hosp, Dept Rheumatol, London, England
[4] UCL, Ctr Rheumatol, London, England
[5] UCL, Dept Neuromuscular Dis, London, England
关键词
Dermatomyositis; Myositis; Phenotype; Interstitial lung disease; Genetic predisposition; Autoantibodies; Immunosuppression; INTERSTITIAL LUNG-DISEASE; CLINICALLY AMYOPATHIC DERMATOMYOSITIS; IMMOBILIZED FIBER COLUMN; ANTI-MDA5; ANTIBODY; JAPANESE PATIENTS; JUVENILE DERMATOMYOSITIS; SINGLE-CENTER; RNA HELICASE; AUTOANTIBODIES; ASSOCIATION;
D O I
10.1007/s00296-021-04819-1
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with Rapidly Progressive Interstitial Lung Disease (RP-ILD) and unique cutaneous features. We reviewed PubMed, SCOPUS and Web of Science databases and selected 87 relevant articles after screening 1485 search results, aiming to gain a better understanding of the pathophysiology, clinical features, diagnosis, and treatment approaches of anti-MDA-5 DM described in the literature. The etiopathogenesis is speculatively linked to an unidentified viral trigger on the background of genetic predisposition culminating in an acquired type I interferonopathy. The clinical phenotype is highly varied in different ethnicities, with new clinical features having been recently described, expanding the spectrum of cases that should raise the suspicion of anti-MDA-5 DM. Unfortunately, the diagnosis is frequently missed despite excessive mortality, calling for wider awareness of suspect symptoms. RP ILD is the major determinant of survival, treatment being largely based on observational studies with recent insights into aggressive combined immunosuppression at the outset.
引用
收藏
页码:1021 / 1036
页数:16
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