title: Marked Improvement with Pirfenidone in a Patient with Idiopathic Pulmonary Fibrosis.
abstract: A man in his mid-60's with idiopathic pulmonary fibrosis and hepatitis B-related liver cirrhosis developed exertional dyspnea and a dry cough lasting for three months. High-resolution computed tomography (HRCT) showed increasing bilateral ground-glass opacity superimposed on the usual interstitial pneumonia pattern. Six months after starting pirfenidone therapy, the partial pressure of arterial oxygen at rest increased from 81 to 101 torr, the predicted forced vital capacity (FVC) value increased from 75% to 94% and the ground-glass opacity on HRCT improved. The FVC value was subsequently maintained near or above baseline for 43 months. We concluded that our patient was a super-responder to pirfenidone therapy.
authors.affiliation: Department of Respiratory Medicine, Respiratory Center, Toranomon Hospital, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Miyamoto
authors.forename: Nasa
authors.initials: N
authors.lastname: Morokawa
authors.forename: Yui
authors.initials: Y
authors.lastname: Takahashi
authors.forename: Kazumasa
authors.initials: K
authors.lastname: Ogawa
authors.forename: Makiko
authors.initials: M
authors.lastname: Takeyasu
authors.forename: Kyoko
authors.initials: K
authors.lastname: Murase
authors.forename: Shigeo
authors.initials: S
authors.lastname: Hanada
authors.forename: Hironori
authors.initials: H
authors.lastname: Uruga
authors.forename: Sayaka
authors.initials: S
authors.lastname: Mochizuki
authors.forename: Hisashi
authors.initials: H
authors.lastname: Takaya
authors.forename: Atsuko
authors.initials: A
authors.lastname: Kurosaki
authors.forename: Kazuma
authors.initials: K
authors.lastname: Kishi
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D003371:Cough / Q000209:etiology; D018450:Disease Progression; D004417:Dyspnea / Q000209:etiology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011728:Pyridones / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone