A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis

This study aimed to investigate whether there was a difference in outcome r elated to histologic pattern in cryptogenic fibrosing alveolitis (CFA) and to see whether there were correlations between clinical and radiologic find ings and histology. One hundred thirteen lung biopsies from consecutive pat ients taken for the diagnosis of diffuse lung disease were reviewed and rec lassified using the Katzenstein and Myers criteria for interstitial pneumon ias. Patients lacking full investigational data at presentation and those w ith conditions predisposing to lung fibrosis were excluded, leaving 15 pati ents diagnosed with nonspecific interstitial pneumonia (NSIP) and 15 with u sual interstitial pneumonia (UIP). Clinical and radiologic findings at pres entation and serial lung function information and survival status in Novemb er 1998 were compared for the two groups. Survival was found to be signific antly greater in the NSIP group compared with the UIP group (p < 0.001). Th is could not be explained by differences in treatment. Patients with UIP sh owed a progressive deterioration in lung function whereas those with NSIP r emained stable. CT scans of patients with UIP showed more fibrosis than tho se of patients with NSIP (p < 0.011). A histologic diagnosis of NSIP is ass ociated with a better prognosis than UIP. This subclassification of CFA is clinically useful.