We describe the clinical courses of the 3 fatal patients (2 females and 1 m
ale) with idiopathic non-specific interstitial pneumonia (NSIP) among 24 pa
tients with NSIP. Lung biopsies were diagnosed to be NSIP group II in all p
atients. The clinical courses from onset to death of these 3 patients were
41 months, 46 months, and 91 months. A follow-up chest CT demonstrated no a
pparent honey-comb formation. We found that i) about 20% of patients with N
SIP died of respiratory failure, ii) in the chest CT findings, apparent hon
ey-comb formation was rare even just before death, iii) prediction of the p
rognosis based on the histological findings was difficult, This is the firs
t report to describe the clinical features of deceased patients with idiopa
thic NSIP; the incidence of fatal cases was considered to range from 10 to
20%.