Pneumonia, Nonspecific Interstitial

Clinical presentation and features are similar to usual interstitial pneumonia (UIP) but with distinct pathologic findings and better prognosis

The median survival is > 10 years

Onset at age 45–55 with slight female predominance

Presents as dyspnea and cough over months; otherwise, presentation is similar to that of UIP

Radiographically, may be indistinguishable from UIP

High-resolution CT finding typically shows bilateral areas of ground-glass attenuation and fibrosis with rare honeycombing

Biopsy shows varying degrees of patchy inflammation and fibrosis, which are uniform in time. Honeycombing is scant

See Table 9–17

Corticosteroids are thought to be effective, but no prospective clinical studies have been published

Prognosis is good overall, but it depends on the degree of fibrosis at diagnosis