RT Journal Article
SR Electronic
T1 Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 143
OP 150
DO 10.1183/09031936.06.00114004
VO 27
IS 1
A1 D. S. Kim
A1 J. H. Park
A1 B. K. Park
A1 J. S. Lee
A1 A. G. Nicholson
A1 T. Colby
YR 2006
UL http://erj.ersjournals.com/content/27/1/143.abstract
AB Although acute exacerbations of idiopathic pulmonary fibrosis are well recognised, there are no studies documenting their prevalence or identifying pre-existing risk factors. This study analysed the clinical, radiological and pathological data of 11 patients who satisfied the criteria for acute exacerbation among 147 patients with biopsy-proven idiopathic pulmonary fibrosis. There were five additional patients who had similar demographics, radiology and surgical lung biopsy pathology, but had clinically less severe disease, and so were not included. The 2-yr frequency of acute exacerbation was 9.6% after the diagnosis. Most exacerbations were idiopathic, although two cases presented after surgical lung biopsy and one after bronchoalveolar lavage. No significant risk factor was found by univariate proportional hazard analysis. Imaging revealed diffuse bilateral ground-glass opacification superimposed on subpleural reticular and honeycombing densities. The biopsies of four patients taken during acute exacerbation exhibited diffuse alveolar damage superimposed upon usual interstitial pneumonia. The findings of this study demonstrate that acute exacerbation of idiopathic pulmonary fibrosis is rather common and this exacerbation is likely to have a spectrum of severity.