Pneumonia, Nonspecific Interstitial

Key Features

• 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

Clinical Findings

• Onset at age 45–55 with slight female predominance

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

Diagnosis

• 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

Treatment

• 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