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For further information, see CMDT Part 9-21: Diffuse Interstitial Pneumonias

Key Features

  • Presentation similar to usual interstitial pneumonitis (UIP) but occurs in younger patients

  • Patients are invariably heavy smokers

  • Usually occurs without symptoms or physiologic evidence of lung disease

  • Prognosis is better than in UIP; median survival > 10 years

Clinical Findings

  • Age 40–45 years

  • Insidious dry cough with exertional dyspnea over months to years

  • Diffuse parenchymal infiltrates

Diagnosis

  • High-resolution CT shows nodular or reticulonodular pattern, more likely to reveal diffuse ground-glass opacities

  • Honeycombing is rare

  • May also show upper lobe emphysema

Treatment

  • Spontaneous remission occurs in up to 20% of patients

  • Smoking cessation is crucial

  • Corticosteroids thought to be effective, but there are no randomized clinical trials to support this view

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