Purpose To examine the serum concentrations of Progranulin (PGRN) and activin A in patients with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) in a pilot study. Methods Twenty-one patients with AE-IPF were compared with 23 patients with stable idiopathic pulmonary fibrosis (IPF) as a control group. Serum PGRN and activin A levels, arterial blood gas measurements and lung function were determined in these two groups. Students t-test was used to compare the differences in PGRN and activin A levels between the two groups, and Spearman correlation coefficient was used to examine the relationship between serum PGRN and activin A levels with carbon monoxide diffusion capacity in patients with IPF. Results Peripheral blood PGRN and activin A levels in patients with AE-IPF were 83.7+10.0 and 14.2+1.7 ng/ml (mean+SD), respectively, higher than those in the control group 61.0+5.8, and 5.8+1.0 (p<0.001). PGRN and activin A levels were significantly negatively correlated with carbon monoxide diffusion capacity (r=-0.857 p<0.001) and r=-0.757 (p<0.001). Conclusion: PGRN and activin A may be involved in the pathogenesis of AE-IPF. They may be possible markers of activity in AE-IPF.