Pirfenidone Improves Familial Idiopathic Pulmonary Fibrosis without Affecting Serum Periostin Levels

Medicina (Kaunas). 2019 May 17;55(5):161. doi: 10.3390/medicina55050161.

Abstract

Background: Antifibrotic agents have been approved for the treatment of idiopathic pulmonary fibrosis (IPF). However, the efficacy of these drugs in the treatment of familial IPF (FIPF) has not been previously reported. Case presentation: We report the case of a 77-year-old man with FIPF, successfully treated with pirfenidone. His uncle died due to IPF, and his niece was diagnosed with the disease. He had worsening dyspnea two months prior to admission to our hospital. Upon admission, he had desaturation when exercising and broad interstitial pneumonia. Administration of pirfenidone improved his dyspnea, desaturation, and the reticular shadow on his chest radiograph. Increased fibrotic marker levels KL-6 and SP-D were also normalized in six months; treatment had no effect on his serum periostin level. Pirfenidone has been effective for over two years. Conclusion: Antifibrotic agents such as pirfenidone may be useful for the management of FIPF, as well as cases of sporadic IPF.

Keywords: familial; idiopathic interstitial pneumonia; idiopathic pulmonary fibrosis; periostin; pirfenidone.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Anti-Inflammatory Agents, Non-Steroidal / pharmacology
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Cell Adhesion Molecules / analysis*
  • Cell Adhesion Molecules / blood
  • Cough / etiology
  • Disease Progression
  • Dyspnea / etiology
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Male
  • Mucin-1 / analysis
  • Mucin-1 / blood
  • Pulmonary Surfactant-Associated Protein A / analysis
  • Pulmonary Surfactant-Associated Protein A / blood
  • Pulmonary Surfactant-Associated Protein D / analysis
  • Pulmonary Surfactant-Associated Protein D / blood
  • Pyridones / pharmacology*
  • Pyridones / therapeutic use

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Cell Adhesion Molecules
  • MUC1 protein, human
  • Mucin-1
  • POSTN protein, human
  • Pulmonary Surfactant-Associated Protein A
  • Pulmonary Surfactant-Associated Protein D
  • Pyridones
  • pirfenidone