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ID 115454
Title Alternative
Super-responder to pirfenidone therapy in IPF
Author
Imakura, Takeshi Tokushima University
Nishimura, Haruka Tokushima University
Takahashi, Naoki Tokushima University
Murakami, Kojin Tokushima University
Keywords
idiopathic pulmonary fibrosis
pirfenidone
chronic liver disease
ground-glass opacity
super-responder
Content Type
Journal Article
Description
Background : Pirfenidone (PFD), an anti-fibrosis drug for idiopathic pulmonary fibrosis (IPF), suppresses disease progression and delays decline of forced vital capacity. However, this drug rarely makes marked improvement of pulmonary function, chest high-resolution computed tomography (HRCT) findings and hypoxia. Case presentation : A 59 year-old-man, who was a former smoker and had a history of alcoholic liver cirrhosis, developed exertional dyspnea and was referred to our hospital. HRCT showed honeycomb changes with surrounding ground-glass opacity (GGO) in a predominantly basal and subpleural distribution. He was diagnosed with IPF and the treatment with PFD was started. At 16 months after the start of treatment, the predicted forced vital capacity value markedly improved from 82.9% to 98.6%. His resting-state partial pressure of arterial oxygen while breathing room air increased from a minimum of 54.7 mmHg (at 2 months treatment) to 72.5 mmHg. The GGO observed at diagnosis disappeared in HRCT. But after 32 months of treatment, his general condition got worse gradually, and he died from chronic progression of IPF after 48 months of treatment. Conclusion : Our case suggests that a complication of chronic liver disease and the existence of GGO may be characteristics of super-responder to PFD treatment for IPF patients.
Journal Title
The Journal of Medical Investigation
ISSN
13496867
13431420
NCID
AA11166929
Publisher
Tokushima University Faculty of Medicine
Volume
67
Issue
3-4
Start Page
358
End Page
361
Sort Key
358
Published Date
2020-08
DOI (Published Version)
URL ( Publisher's Version )
FullText File
language
eng
TextVersion
Publisher
departments
University Hospital
Medical Sciences