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Clinical Course of Probable Idiopathic Pulmonary Fibrosis  

Kyung, Sun Young (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
Park, Cheul Hee (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
Lim, Young-Hee (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
An, Chang Hyeok (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
Lee, Sang Pyo (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
Park, Jeong Woong (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
Jeon, Kyeongman (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center)
Lee, Byoung-Hoon (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center)
Chung, Man Pyo (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center)
Jeong, Sung Hwan (Division of Pulmonology, Department of Internal Medicine, Gachon Medical School, Gil Medical Center)
Publication Information
Tuberculosis and Respiratory Diseases / v.59, no.1, 2005 , pp. 77-85 More about this Journal
Abstract
Background : According to the 2002 consensus report of the American Thoracic Society/European Respiratory Society (ATS/ERS), idiopathic pulmonary fibrosis (IPF) was classified as biopsy proven or probable IPF. Probable IPF is defined as those with distinctive features that allow for a confident diagnosis of IPF/usual interstitial pneumonia (UIP) within an appropriate clinical setting. The determination of the clinical course of probable IPF, as diagnosed by the ATS/ERS criteria, was studied. Methods : Between March 1995 and August 2002, 36 patients with probable IPF, from two tertiary referral hospitals, were enrolled in this study. The clinical characteristics, prognostic factors and treatment efficacy of these patients were retrospectively evaluated. Results : The mean age of the subjects was $65{\pm}6$ years. The one and 3 year survival rates were 82.4 and 50.3%, respectively, and a median survival period of 42.0 months. The total cell count of bronchoalveolar lavage was higher in the death than the survival group (p<0.05). No survival benefits were found in the cytoxan and steroid treatment groups compared with other treatment groups. Conclusion : These results suggest that the clinical course of probable IPF may be similar to that of biopsy-proven UIP. However, atypical patients must undergo an open lung biopsy for confirmation of the diagnosis.
Keywords
Idiopathic pulmonary fibrosis; Probable IPF;
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