In 65 patients with IPF, both LDH at diagnosis of AE (HR 1.003, 95% CI: 1.001–1.005, p = 0.004) and change in LDH (HR 1.004, 95% CI: 1.001–1.008, p = 0.017) were independent risk factors for mortality in multivariable analysis, while change in KL-6 (HR 1.000, 95% CI: 0.999–1.001, p = 0.197) was not. The gene discussed is MUC1; the disease is idiopathic pulmonary fibrosis.