Clinical Course and Lung function Change of Idiopathic Nonspecific Interstitial Pneumonia

¹ Division of Pulmonary and Critical Care Medicine, University of Ulsan, College of Medicine, Asan Medical Center
² Dept of Radiology, University of Ulsan, College of Medicine, Asan Medical Center
³ Dept of Rheumatology, University of Ulsan, College of Medicine, Asan Medical Center
⁴ Dept of Pathology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea
⁵ Laboratory and Anatomic Pathology, National Hospital Organization Kinki-chuo Chest Medical Center, Osaka, Japan
⁶ Dept of Histopathology, Royal Brompton Hospital, London, UK
⁷ Dept of Pathology, Mayo clinic, Scottsdale USA

^* To whom correspondence should be addressed. E-mail: dskim{at}amc.seoul.kr.

	Abstract

Most studies of idiopathic nonspecific interstitial pneumonia (NSIP) looked at mortality. To clarify the detailed outcome and prognostic markers in idiopathic NSIP, we analyzed the clinical course with initial radiologic and clinical features.

The clinical course of 83 patients who were classified as idiopathic NSIP (72 fibrotic: 11 cellular, male: female=27:56, mean age 54.4±10.1 years) were retrospectively analyzed. In fibrotic NSIP, 16 patients (22%) died of NSIP-related causes during the median follow-up of 53 (range 0.3–181) months. Despite the favorable survival (5 year. 74%), patients with fibrotic NSIP were frequently hospitalized with recurrence rate of 36%. Reduced FVC at 12 months was a predictor of mortality. On follow-up, lung function was improved or stable in about 80% of the patients. The extent of consolidation and ground glass opacity on initial HRCT was significantly correlated with serial changes of lung function and the presence of honeycombing was a predictor of poor prognosis. During follow up, 8 patients (10%) developed collagen vascular disease (CVD) later. As a conclusion, the overall prognosis of fibrotic NSIP was good; however, there were significant recurrences despite initial improvement and a subset of the patients did not respond to therapy. Some of patients developed CVD later.