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

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Yukiko Miura
Takefumi Saito
Kazutaka Fujita
Toru Tanaka
Yoshiya Tsunoda
Arata Azuma
Takahito Nei
Yohei Yatagai
Shigen Rin
Akimasa Sekine
Kenji Hayashihara

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.

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