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Physiology Is a Stronger Predictor of Survival than Pathology in Fibrotic Interstitial Pneumonia

Division of Pulmonary and Critical Care Medicine, Department of Medicine, and Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea; Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC; Laboratory of Anatomical Pathology, Kyoto University Hospital, Kyoto, Japan; and Department of Pathology, Mayo Clinic, Scottsdale, Arizona

Correspondence and requests for reprints should be addressed to Dong Soon Kim, M.D., Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnap Dong Songpa Ku, Seoul 138-736, South Korea. E-mail: dskim{at}amc.seoul.kr

The histopathologic pattern provides the most important prognostic marker for idiopathic interstitial pneumonia; however, studies have suggested that short-term changes in lung function may be more important. We investigated the prognostic factors for fibrotic interstitial pneumonia. The clinical features and follow-up course of 179 patients (131 with idiopathic pulmonary fibrosis and 48 with nonspecific interstitial pneumonia; 41 fibrotic types and 7 cellular) were analyzed retrospectively. The lung function indices improved or stabilized in most patients with fibrotic nonspecific interstitial pneumonia in contrast to the deterioration or stable condition of most patients with idiopathic pulmonary fibrosis. The 5-year survival of patients with fibrotic nonspecific interstitial pneumonia (76.2%) was better than for those with idiopathic pulmonary fibrosis (43.8%) (p = 0.007). Multivariate analysis at the time of presentation revealed that pathologic pattern, age, and diffusion capacity had important prognostic implications. However, after 6 months of follow-up, changes in FVC, initial diffusion capacity, and sex were the only independent prognostic factors, with no additional prognostic information conferred by the histologic diagnosis. Our data confirmed the importance of physiological parameters including short-term change in FVC. However, at the time of diagnosis, histopathology was important for the prediction of prognosis and future change in lung function.

Key Words: fibrotic nonspecific interstitial pneumonia • idiopathic pulmonary fibrosis • prognostic factor • pulmonary function • surgical lung biopsy

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