JOURNAL ARTICLE

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

Yukiko Miura, Takefumi Saito, Kazutaka Fujita, Yoshiya Tsunoda, Toru Tanaka, Hiroyuki Takoi, Yohei Yatagai, Shigen Rin, Akimasa Sekine, Kenji Hayashihara, Takahito Nei, Arata Azuma
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG 2014 October 20, 31 (3): 235-8
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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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