title: A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone.
abstract: The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. High-resolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.
authors.affiliation: Department of Respiratory Medicine & Rheumatology, Institute of Biomedical Sciences, Tokushima University Graduate School, Japan.
authors.forename: Seidai
authors.initials: S
authors.lastname: Sato
authors.forename: Masaki
authors.initials: M
authors.lastname: Hanibuchi
authors.forename: Mikiko
authors.initials: M
authors.lastname: Takahashi
authors.forename: Yuh
authors.initials: Y
authors.lastname: Fukuda
authors.forename: Shun
authors.initials: S
authors.lastname: Morizumi
authors.forename: Yuko
authors.initials: Y
authors.lastname: Toyoda
authors.forename: Hisatsugu
authors.initials: H
authors.lastname: Goto
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Nishioka
mesh_terms: D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage*; D004417:Dyspnea / Q000209:etiology / Q000473:pathology*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D008168:Lung / Q000473:pathology*; D008297:Male; D010995:Pleural Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D011728:Pyridones / Q000008:administration & dosage*; D013902:Radiography, Thoracic; D012143:Respiratory Physiological Phenomena / Q000187:drug effects; D013997:Time Factors; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects; D015431:Weight Loss
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone