Low serum KL-6 and SP-D levels, high serum AST and γ-GTP levels, high CD4+/CD8+ ratio in BAL fluid, and predominance of subpleural GGO or irregular linear opacity in HRCT may help to discriminate anti-MDA-5 antibody-positive CADM-ILD with poor prognosis. Here, MUC1 is linked to interstitial lung disease.