Premium
Disease progression in idiopathic pulmonary fibrosis without pulmonary function impairment
Author(s) -
Kondoh Yasuhiro,
Taniguchi Hiroyuki,
Ogura Takashi,
Johkoh Takeshi,
Fujimoto Kiminori,
Sumikawa Hiromitsu,
Kataoka Kensuke,
Baba Tomohisa,
Colby Thomas V.,
Kitaichi Masanori
Publication year - 2013
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/resp.12082
Subject(s) - medicine , honeycombing , dlco , pulmonary function testing , idiopathic pulmonary fibrosis , vital capacity , odds ratio , diffusing capacity , asymptomatic , interstitial lung disease , surgery , lung , lung function
Abstract Background and objective Despite significant recent progress in the understanding of idiopathic pulmonary fibrosis ( IPF ), the early phase of the disease is still poorly understood. We studied patients with IPF without pulmonary function impairment in order to determine the clinical features, natural history and key findings for physiological progression. Methods Twenty‐five patients without pulmonary function impairment were identified from among patients with previously diagnosed IPF who underwent surgical lung biopsies between J anuary 1997 and D ecember 2006 at our institutions. ‘Without pulmonary function impairment’ was defined as both forced vital capacity ( FVC ) and diffusing capacity for carbon monoxide ( DLco ) >80% predicted. Patients diagnosed with IPF through multidisciplinary discussion based on the new IPF guidelines were the subjects of this study. Results Sixteen patients had a confirmed diagnosis of IPF . Eleven patients presented with chest X ‐ray abnormality found during an annual health examination. Seven patients were asymptomatic. Eleven patients showed physiological disease progression (median time; 19.9 ± 12.3 months) defined by a decline of at least 10% in FVC or at least 15% in DLco . Univariate analysis revealed that both usual interstitial pneumonia pattern and extent of honeycombing on high‐resolution computed tomography (HRCT) were factors associated with disease progression (odds ratio 5.634, 95% confidence interval 1.364–23.278; odds ratio 2.371/5%, 95% confidence interval 1.042–5.395). Conclusions IPF patients without pulmonary function impairment have a progressive but slow clinical course. The existence and extent of honeycombing on HRCT are predictive of disease progression.