Idiopathic Pulmonary Fibrosis

IPF, which is also known as usual interstitial pneumonia (UIP), is the most common idiopathic interstitial pneumonia. Cigarette smoking is a risk factor for IPF. Common respiratory symptoms include exertional dyspnea and a nonproductive cough. Physical examination is notable for inspiratory crackles at the lung bases. Clubbing may occur. High-resolution chest CT scans show subpleural reticular opacities predominantly in the lower lung fields, which are associated with honeycombing in advanced disease. Surgical lung biopsy is usually required to confirm the diagnosis, although pts with classic UIP patterns on CT scan may not require a biopsy. IPF can include acute exacerbations characterized by accelerated clinical deterioration over days to weeks. IPF is poorly responsive to available pharmacologic treatment, although recent studies have suggested benefit from pirfenidone and nintedanib.