Progranulin And Activin A Concentrations Are Elevated In Serum From Patients With Acute Exacerbations of Idiopathic Pulmonary Fibrosis

Abstract

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.

Progranulin Promotes the Formation and Development of Capsules Caused by Silicone in Sprague-Dawley Rats

“…PGRN can activate multiple pathways in various tumors, such as ERK and TNFR2/Akt signalling, 15 PI3K 16 or Wnt signalling 17 to stimulate the proliferation and invasion of tumor entities. 18,19 In addition to its role in tumors, PGRN is also involved in various fibrotic and immune system diseases (wound healing, 20,21 scleroderma, 22 pulmonary fibrosis 23 and rheumatoid arthritis 24 ).Moreover, changes in serum PGRN levels reflect the development and outcome of certain diseases. 25,26 Formation of an implant capsule is a typical repair and fibrosis process after surgical injury.…”