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Copyright ©ERS Journals Ltd 2006
Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features
1 Dept of Pulmonary and Critical Care Medicine, and 2 Dept of Radiology, Asan Medical Center, Ulsan University, Seoul, South Korea. 3 Dept of Pathology, Royal Brompton Hospital, London, UK. 4 Dept of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, Arizona, USA.
CORRESPONDENCE: D. S. Kim, Dept of Pulmonary and Critical Care Medicine, Asan Medical Center, Ulsan University, Poongnap-dong, Songpa-ku, Seoul, Korea. Fax: 82 230106968. E-mail: dskim{at}amc.seoul.kr
Keywords: Acute exacerbation, diagnostic criteria, frequency, idiopathic pulmonary fibrosis, risk factors, surgical lung biopsy pathology
Received: October 2, 2004
Accepted September 9, 2005
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.
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