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For further information, see CMDT Part 9-22: Diffuse Interstitial Pneumonias

KEY FEATURES

  • Presentation similar to usual interstitial pneumonia (UIP) but it 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

  • Clinical experience suggests that patients frequently respond to corticosteroids and should be given a trial of steroid therapy—typically prednisone, 1–2 mg/kg/d for a minimum of 2 months

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