Pirfenidone Alleviates Inflammation and Fibrosis of Acute Respiratory Distress Syndrome by Modulating the Transforming Growth Factor-β/Smad Signaling Pathway

Int J Mol Sci. 2024 Jul 23;25(15):8014. doi: 10.3390/ijms25158014.

Abstract

Acute respiratory distress syndrome (ARDS) occurs as an acute onset condition, and patients present with diffuse alveolar damage, refractory hypoxemia, and non-cardiac pulmonary edema. ARDS progresses through an initial exudative phase, an inflammatory phase, and a final fibrotic phase. Pirfenidone, a powerful anti-fibrotic agent, is known as an agent that inhibits the progression of fibrosis in idiopathic pulmonary fibrosis. In this study, we studied the treatment efficiency of pirfenidone on lipopolysaccharide (LPS) and bleomycin-induced ARDS using rats. The ARDS rat model was created by the intratracheal administration of 3 mg/kg LPS of and 3 mg/kg of bleomycin dissolved in 0.2 mL of normal saline. The pirfenidone treatment group was administered 100 or 200 mg/kg of pirfenidone dissolved in 0.5 mL distilled water orally 10 times every 2 days for 20 days. The administration of LPS and bleomycin intratracheally increased lung injury scores and significantly produced pro-inflammatory cytokines. ARDS induction increased the expressions of transforming growth factor (TGF)-β1/Smad-2 signaling factors. Additionally, matrix metalloproteinase (MMP)-9/tissue inhibitor of metalloproteinase (TIMP)-1 imbalance occurred, resulting in enhanced fibrosis-related factors. Treatment with pirfenidone strongly suppressed the expressions of TGF-β1/Smad-2 signaling factors and improved the imbalance of MMP-9/TIMP-1 compared to the untreated group. These effects led to a decrease in fibrosis factors and pro-inflammatory cytokines, promoting the recovery of damaged lung tissue. These results of this study showed that pirfenidone administration suppressed inflammation and fibrosis in the ARDS animal model. Therefore, pirfenidone can be considered a new early treatment for ARDS.

Keywords: acute respiratory distress syndrome; bleomycin; inflammation; lipopolysaccharide; pirfenidone; pulmonary fibrosis.

MeSH terms

  • Animals
  • Bleomycin* / adverse effects
  • Disease Models, Animal
  • Inflammation / drug therapy
  • Inflammation / metabolism
  • Inflammation / pathology
  • Lipopolysaccharides*
  • Lung / drug effects
  • Lung / metabolism
  • Lung / pathology
  • Male
  • Matrix Metalloproteinase 9 / metabolism
  • Pyridones* / pharmacology
  • Pyridones* / therapeutic use
  • Rats
  • Rats, Sprague-Dawley
  • Respiratory Distress Syndrome* / chemically induced
  • Respiratory Distress Syndrome* / drug therapy
  • Respiratory Distress Syndrome* / metabolism
  • Respiratory Distress Syndrome* / pathology
  • Signal Transduction* / drug effects
  • Smad Proteins / metabolism
  • Smad2 Protein / metabolism
  • Tissue Inhibitor of Metalloproteinase-1 / metabolism
  • Transforming Growth Factor beta / metabolism
  • Transforming Growth Factor beta1 / metabolism

Substances

  • pirfenidone
  • Pyridones
  • Bleomycin
  • Lipopolysaccharides
  • Tissue Inhibitor of Metalloproteinase-1
  • Smad2 Protein
  • Matrix Metalloproteinase 9
  • Transforming Growth Factor beta1
  • Smad2 protein, rat
  • Transforming Growth Factor beta
  • TIMP1 protein, rat
  • Smad Proteins