A chronic, progressive, and ultimately fatal lung disease characterized by scarring (fibrosis) of the lung tissue without known cause. The fibrosis is irreversible and leads to progressive decline in lung function, making breathing increasingly difficult.

• 1High-resolution CT (HRCT) - honeycombing pattern
• 2Pulmonary function tests (restrictive pattern)
• 3Lung biopsy (if HRCT inconclusive)
• 46-minute walk test
• 5Echocardiogram (pulmonary hypertension assessment)

Poor; median survival 3-5 years without transplant. Antifibrotics may extend life by 1-2 years. Acute exacerbations can be fatal. Early referral for transplant evaluation recommended.

• Smoking cessation
• Avoid occupational exposures
• Treatment of GERD
• Vaccinations (pneumococcal, flu)