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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

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