Successful treatment with tofacitinib for anti-melanoma differentiation-associated gene 5 antibody-positive juvenile dermatomyositis: case reports and review of the literature

Yamazaki, Susumu; Shimizu, Masaki; Yakabe, Ayane; Inage, Eisuke; Jimbo, Keisuke; Suzuki, Mitsuyoshi; Miyaoka, Futaba; Kaneko, Shuya; Irabu, Hitoshi; Shimbo, Asami; Ohtomo, Yoshiyuki; Mori, Masaaki; Morio, Tomohiro; Shimizu, Toshiaki

doi:10.1080/25785826.2024.2336687

Immunological Medicine

2024

DOI: 10.1080/25785826.2024.2336687

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Successful treatment with tofacitinib for anti-melanoma differentiation-associated gene 5 antibody-positive juvenile dermatomyositis: case reports and review of the literature

Susumu Yamazaki,

Masaki Shimizu,

Ayane Yakabe

et al.

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Antimelanoma differentiation antigen 5-positive dermatomyositis: an update

Lu,

Peng,

Wang

2024

Current Opinion in Rheumatology

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Purpose of review Antimelanoma differentiation antigen 5-dermatomyositis (MDA5-DM) is a complex and serious systemic autoimmune disease that primarily affects the skin and lungs. In this review, we aimed to provide new insights into the clinical features, pathogenesis, and practical management approach for this disease. Recent findings Although lung lesions are prominent in most patients with MDA5-DM, they are now recognized as heterogeneous diseases. Peripheral blood lymphocyte count can serve as a simple and reliable laboratory parameter for categorizing MDA5-DM into three subgroups: mild, medium, and severe. Recent studies have implicated viral infection, genetic factors, autoimmunity against MDA5, multiple immune cells, and interferons as significant contributors to MDA5-DM pathogenesis. In addition to traditional treatments with glucocorticoids and immunosuppressants, many new approaches, including new biologics and targeted agents, have been explored. Additionally, infection is a common complication of MDA5-DM, and prophylaxis or treatment of the infection is as important as treating the primary disease. Summary Knowledge of clinical characteristics and pathogenesis of MDA5-DM has grown in recent years. Although many new therapeutic approaches have been explored, further studies are required to confirm their efficacy.

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Antimelanoma differentiation antigen 5-positive dermatomyositis: an update

Lu,

Peng,

Wang

2024

Current Opinion in Rheumatology

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Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases—A Narrative Review

Kobayashi

2024

Children

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Juvenile idiopathic inflammatory myopathy (JIIM) is a rare systemic autoimmune disease characterized by skeletal muscle weakness with or without a skin rash. Juvenile dermatomyositis (JDM) is the most common subtype of JIIM, accounting for 80% of JIIM. Recent studies identified several myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs). Each MSA or MAA is associated with distinct clinical features and outcomes, although there are several differences in the prevalence of MSA/MAA and autoantibody–phenotype relationships between age and ethnic groups. Histopathological studies have revealed critical roles of type I interferons and vasculopathy in the development of JDM. Serological classification mostly corresponds to clinicopathological classification. Novel therapeutic agents, such as biologics and Janus kinase inhibitors (JAKi), have been developed; however, to date, there is a lack of high-level evidence. As advances in treatment have reduced the mortality rate of JIIM, recent studies have focused on medium- and long-term outcomes. However, rapidly progressive interstitial lung disease (RP-ILD) remains a major cause of death in anti-melanoma differentiation gene 5 autoantibody-positive JDM. Early diagnosis and intervention using a multi-drug regimen is critical for the treatment of RP-ILD. Rituximab and JAKi may reduce mortality in patients with JDM-associated RP-ILD refractory to conventional therapy.

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Successful treatment with tofacitinib for anti-melanoma differentiation-associated gene 5 antibody-positive juvenile dermatomyositis: case reports and review of the literature

Cited by 2 publications

References 44 publications

Antimelanoma differentiation antigen 5-positive dermatomyositis: an update

Antimelanoma differentiation antigen 5-positive dermatomyositis: an update

Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases—A Narrative Review

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