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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
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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
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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