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Sarcoidosis Vasc Diffuse Lung Dis. 2014 Oct 20;31(3):235-8.

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

Author information

1
Department of Respiratory Medicine, National Hospital Organization Ibarakihigashi Hospital, Japan; Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Japan. s7081@nms.ac.jp.

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.

PMID:
25363224
[Indexed for MEDLINE]

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