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.