Diagnosis and Management of Idiopathic Pulmonary Fibrosis

Clinical Features and Diagnosis

The European Respiratory Society recommends that a diagnosis of idiopathic pulmonary fibrosis (IPF) should be considered in patients over 60 years old with a history of progressive exertional dyspnea and nonproductive cough, and fine bibasilar inspiratory crackles on physical examination 3, 4, 5
The American Thoracic Society suggests that high-resolution CT (HRCT) is mandatory to confirm the diagnosis of IPF, and should demonstrate a usual interstitial pneumonia (UIP) pattern with subpleural and basal predominant distribution, honeycombing with or without peripheral traction bronchiectasis 9, 1, 8, 2
If HRCT shows a definite UIP pattern in the appropriate clinical context, IPF can be diagnosed without surgical lung biopsy, according to the European Respiratory Society 3, 4, 5

The American College of Chest Physicians recommends excluding chronic obstructive pulmonary disease (COPD) by the presence of bilateral inspiratory crackles, increased reticular markings on chest x-ray, and nonproductive cough, which are not typical of COPD 7, 8, 6
Heart failure should be excluded by the absence of peripheral edema, and the presence of nonproductive cough with fine crackles, which are more consistent with IPF than heart failure 6, 7

The American Thoracic Society states that untreated IPF has a poor prognosis, with a median survival of 3-5 years from diagnosis, but antifibrotic therapy (pirfenidone or nintedanib) can reduce 1-year mortality and slow disease progression 1
Early diagnosis and treatment initiation are crucial for improving outcomes in IPF, according to the European Respiratory Society 3, 4, 5