Perspectives in the treatment of interstitial lung disease accompanied with anti‐melanoma differentiation‐associated gene 5‐positive dermatomyositis

Abstract

The establishment of an effective treatment for rapidly progressive interstitial lung disease (RP-ILD) accompanied with anti-melanoma differentiation-associated gene 5 (MDA5)-positive dermatomyositis is important. [1][2][3] As the disease is rare and progresses rapidly with a poor prognosis, it is difficult to conduct a clinical study with a high level of evidence, such as a randomized control trial. Despite these limitations, evidence on the treatment of this disease, such as the efficacy of Janus kinase (JAK) inhibitors, plasmapheresis, and the triple immunosuppressive therapy with glucocorticoids (GCs), calcineurin inhibitors (CNIs), and intravenous cyclophosphamide (IVCY), is growing. [4][5][6][7] Here, we describe the treatment strategies and future prospects for this disease. | IMP ORTAN CE OF E ARLY D IAG NOS IS IN RP-ILDSince ILD often progresses rapidly with an acute or subacute course and presents with diffuse alveolar damage, diagnosis and treatment need to be initiated before the disease progresses. Typical lung imaging findings, clinically amyopathic dermatomyositis (CADM), and hyperferritinemia are important for diagnosis. Subsequently, confirmation of anti-MDA5 antibody, which is detected using immunoprecipitation, line blotting, or enzyme-linked immunosorbent assay, is also important. 8,9 Lung involvement has a characteristic clinical course and imaging findings. The intervals between the onset of cutaneous symptoms