Abstract

I.N. Park, Y. Jegal, D.S. Kim, K-H. Do, B. Yoo, T.S. Shim, C-M. Lim, S.D. Lee, Y. Koh, W.S. Kim, W.D. Kim, S.J. Jang, M. Kitaichi, A.G. Nicholsone T.V. Colby

Division of Pulmonary and Critical Care Medicine, Dept of Radiology, Dept of Rheumatology, and Dept of Pathology, University of Ulsan, College of Medicine, Asan Medical Center, Seoul, Korea, Laboratory and Anatomic Pathology, National Hospital Organization Kinkichuo Chest Medical Center, Osaka, Japan, Dept of Histopathology, Royal Brompton Hospital, London, UK, Dept of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ, USA.

Contact: D.S. Kim dskim@amc.seoul.kr, Division of Pulmonary and Critical Care Medicine University of Ulsan College of Medicine Asan Medical Center, 388-1 Pungnap-2dong Songpa-gu Seoul 138-736 Korea Fax: 82 230106968

ABSTRACT

Most studies of idiopathic nonspecific interstitial pneumonia (NSIP) have primarily studied mortality. In order to clarify the detailed outcome and prognostic markers in idiopathic NSIP, the clinical course with initial radiological and clinical features was analysed. The clinical course of 83 patients who were classified with idiopathic NSIP (72 fibrotic, 11 cellular; 27 males and 56 females; mean¡SD age 54.4±10.1 yrs) was retrospectively analysed. In fibrotic NSIP, 16 (22%) patients died of NSIP-related causes with a median (range) followup of 53 (0.3-181) months. Despite the favourable survival (5-yr 74%), patients with fibrotic NSIP were frequently hospitalised with recurrence rate of 36%. Reduced forced vital capacity at 12 months was a predictor of mortality. On follow-up, lung function was improved or stable in >80% of the patients. The extent of consolidation and sticla mata opacity on initial high resolution computed tomography

correlated significantly with serial changes of lung function, and the presence of aspect de fagure de miere was a predictor of poor prognosis. During follow-up, eight (10%) patients developed collagen vascular disease.

In conclusion, the overall prognosis of fibrotic nonspecific interstitial pneumonia was good; however, there were significant recurrences despite initial improvement and a subset of the patients did not respond to therapy. Some patients developed collagen vascular diseases at a later date.

Keywords: Clinical course, fibrotic nonspecific interstitial pneumonia, pulmonary function test, recurrence