Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory muscle diseases. Disease-specific antibodies—such as anti-aminoacyl-tRNA synthetase (including anti-Jo-1), anti-MDA5, and anti-TIF1-γ—are associated with distinct clinical phenotypes. Rapidly progressive interstitial pneumonia linked to anti-MDA5–positive DM has a poor prognosis: although combined immunosuppressive therapy has improved 2-year survival, 25% of patients still die from respiratory failure within six months of onset [1].

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Dermatomyositis and Polymyositis

  • Hiroko Kanda

摘要

Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory muscle diseases. Disease-specific antibodies—such as anti-aminoacyl-tRNA synthetase (including anti-Jo-1), anti-MDA5, and anti-TIF1-γ—are associated with distinct clinical phenotypes. Rapidly progressive interstitial pneumonia linked to anti-MDA5–positive DM has a poor prognosis: although combined immunosuppressive therapy has improved 2-year survival, 25% of patients still die from respiratory failure within six months of onset [1].