Anti-MDA5 antibody-positive dermatomyositis: pathogenesis and clinical progress

Anti-melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (MDA5-DM) is a subtype of dermatomyositis. Although the aetiology and pathology remain unclear, increasing evidence suggests that viral infection is a potential trigger of MDA5-DM. Multiple factors, including...

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Published inNature reviews. Rheumatology Vol. 20; no. 1; pp. 48 - 62
Main Authors Lu, Xin, Peng, Qinglin, Wang, Guochun
Format Journal Article
LanguageEnglish
Published United States Nature Publishing Group 01.01.2024
Subjects
Antibodies
Autoantibodies
Biomarkers
Cell activation
Dermatomyositis
Dermatomyositis - complications
Ferritin
Humans
Interferon-Induced Helicase, IFIH1
Leukocytes (neutrophilic)
Lung diseases
Lung Diseases, Interstitial - diagnosis
Lung Diseases, Interstitial - etiology
Lymphocytes B
Lymphocytes T
Lymphopenia
Macrophages
Melanoma
Pathogenesis
Pathophysiology
Patients
Prognosis
Retrospective Studies
Serum levels
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Summary:Anti-melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (MDA5-DM) is a subtype of dermatomyositis. Although the aetiology and pathology remain unclear, increasing evidence suggests that viral infection is a potential trigger of MDA5-DM. Multiple factors, including T cells, B cells, neutrophils and macrophages, are implicated in the pathophysiology of MDA5-DM. Distinctive skin rashes, rapidly progressive interstitial lung disease, peripheral lymphopenia and elevated serum ferritin levels are the most prominent clinical and laboratory features of MDA5-DM. Concomitant infection is a common complication of MDA5-DM. The proper evaluation of patients with MDA5-DM requires knowledge of the disease heterogeneity and clinical course variability. Several biomarkers, including serum levels of anti-MDA5 antibodies and biomarkers related to macrophage activation, have been identified as useful tools for monitoring disease activity and prognosis. MDA5-DM shows a poor response to conventional glucocorticoid and immunosuppressant therapy and has a poor overall prognosis. Therefore, there is an urgent need to explore the key pathogenic mechanisms of MDA5-DM and develop novel therapeutic options for patients. This Review discusses recent clinical progress and pathogenic findings of MDA5-DM.
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ISSN:1759-4790
1759-4804
DOI:10.1038/s41584-023-01054-9