Pathogenesis and Treatment of Interstitial Lung Disease Accompanied by Anti-melanoma Differentiation-associated Gene 5-positive Idiopathic Inflammatory Myopathies

Interstitial lung disease (ILD) progresses rapidly and presents with diffuse alveolar damage for patients with anti-melanoma differentiation-associated gene 5 (MDA5)-positive idiopathic inflammatory myopathies. The activation of monocytes/macrophages, B cells, and interferonopathy appears to be involved in the pathogenesis of the disease and can be a therapeutic target. An early diagnosis and intensive immunosuppressive treatment at an early stage are effective for the treatment of ILD. Evidence on treatment, such as the efficacy of Janus kinase inhibitors, rituximab, plasmapheresis, and triple immunosuppressive therapy with glucocorticoids, calcineurin inhibitors, and intravenous cyclophosphamide, has been growing. Careful monitoring of infections and adverse events during intensive immunosuppressive treatment is necessary. It is desirable to elucidate the pathogenesis of the disease, identify indications for intensive treatment, and provide appropriate therapy.