title: Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.
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.
authors.affiliation: 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.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Miura
authors.forename: Takefumi
authors.initials: T
authors.lastname: Saito
authors.forename: Kazutaka
authors.initials: K
authors.lastname: Fujita
authors.forename: Yoshiya
authors.initials: Y
authors.lastname: Tsunoda
authors.forename: Toru
authors.initials: T
authors.lastname: Tanaka
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Takoi
authors.forename: Yohei
authors.initials: Y
authors.lastname: Yatagai
authors.forename: Shigen
authors.initials: S
authors.lastname: Rin
authors.forename: Akimasa
authors.initials: A
authors.lastname: Sekine
authors.forename: Kenji
authors.initials: K
authors.lastname: Hayashihara
authors.forename: Takahito
authors.initials: T
authors.lastname: Nei
authors.forename: Arata
authors.initials: A
authors.lastname: Azuma
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D008168:Lung / Q000187:drug effects* / Q000473:pathology / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011728:Pyridones / Q000627:therapeutic use*; D020127:Recovery of Function; D012189:Retrospective Studies; D045743:Scleroderma, Diffuse / Q000150:complications* / Q000175:diagnosis; D012594:Scleroderma, Localized / Q000150:complications* / Q000175:diagnosis; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014797:Vital Capacity
chemical_list: D011728:Pyridones; C093844:pirfenidone