Multivariate Cox proportional hazards model analysis identified that anti-MDA5 antibody (HR 11.639, [95% CI 1.338–101.240], P = 0.026) was an independent risk factor for mortality due to RP-ILD, and lymphocytes at <30% in BALF (HR 12.048, [95% CI 1.466–99.031], P = 0.021) might be associated with poor survival of RP-ILD (Table 5). Here, IFIH1 is linked to retinitis pigmentosa 1.