Clinical features of non-specific interstitial pneumonia

The clinical features of 24 patients with non-specific interstitial pneumon ia (NIP) were evaluated. The patients consisted of seven men and 17 women, with a median age of 60 years. In seven patients, the disease was idiopathi c and eight had collagen vascular diseases. Cough, dyspnoea and fever were frequently observed. The time interval between the onset of symptoms and op en lung biopsy was 3 months. Mild increases of IgG, CRP, and LDH were also frequently observed. The average per cent VC was 65.1 +/- 3.2% and the aver age PaO2 was 71.3 +/- 2.4 Torr. Bronchoalveolar lavage was done in 20 patie nts, and a moderate increase in lymphocytes (27.8 +/- 6.7%) with a low CD4/ CD8 ratio (0.86 +/- 0.22) was observed. Multiple patchy infiltration or dif fuse interstitial shadows, located predominantly in the lower fields of bot h lungs were the characteristic chest CT findings. Lung biopsies in this gr oup were characterized by varying proportions of chronic interstitial infla mmation and fibrosis which was temporarily uniform. Patients were given ste roid pulse therapy or oral steroids. The results were mild to marked improv ements in chest roentgenographic findings and lung functions. Four patients (16.7%) died because of respiratory failure caused by NIP. This is the fir st report to describe clinical features of NIP since the original report by Katzenstein and Fiorelli.