Taken together, patients with anti‐MDA5 Ab‐positive dermatomyositis are prone to suffering from RP‐ILD and the decrease in lymphocyte count is an important risk factor for complicated with RP‐ILD and patient death, which may be a convenient and feasible predictor for anti‐MDA5 Ab‐positive dermatomyositis in Chinese population. This evidence concerns the gene IFIH1 and retinitis pigmentosa 1.