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.
MeSH terms
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Aged
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Female
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Humans
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Lung / drug effects*
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Lung / pathology
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Lung / physiopathology
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Lung Diseases, Interstitial / diagnosis
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Lung Diseases, Interstitial / drug therapy*
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Lung Diseases, Interstitial / etiology
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Lung Diseases, Interstitial / physiopathology
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Male
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Middle Aged
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Pyridones / therapeutic use*
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Recovery of Function
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Retrospective Studies
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Scleroderma, Diffuse / complications*
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Scleroderma, Diffuse / diagnosis
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Scleroderma, Localized / complications*
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Scleroderma, Localized / diagnosis
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Time Factors
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Tomography, X-Ray Computed
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Treatment Outcome
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Vital Capacity