Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease

Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease

Authors

  • Yukiko Miura Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan; Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan
  • Takefumi Saito Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Kazutaka Fujita Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Toru Tanaka Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Yoshiya Tsunoda Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Arata Azuma Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Takahito Nei Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Yohei Yatagai Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Shigen Rin Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Akimasa Sekine Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan
  • Kenji Hayashihara Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Keywords:

Antifibrotic agent, interstitial lung disease, pirfenidone, scleroderma, vital capacity

Abstract

Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.

Author Biographies

Yukiko Miura, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan; Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan

Department of Pulmonary Medicine and Oncology, an assistant professor

Takefumi Saito, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Department of Respiratory Medicine, clinical professor

Kazutaka Fujita, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Department of Respiratory Medicine, MD

Toru Tanaka, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Department of Respiratory Medicine, MD

Yoshiya Tsunoda, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Department of Respiratory Medicine, MD

Arata Azuma, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Department of Pulmonary Medicine and Oncology, professor

Takahito Nei, Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan

Department of Pulmonary Medicine and Oncology, MD

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Published

20-10-2014

Issue

Vol. 31 No. 3 (2014)

Section

Case Series

License

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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How to Cite

1.
Miura Y, Saito T, Fujita K, Tanaka T, Tsunoda Y, Azuma A, et al. Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease. Sarcoidosis Vasc Diffuse Lung Dis [Internet]. 2014 Oct. 20 [cited 2025 May 20];31(3):235-8. Available from: https://mattioli1885journals.com/index.php/sarcoidosis/article/view/3326