Idiopathic pulmonary fibrosis in a smoker with indeterminate usual interstitial pneumonia pattern on high-resolution computed tomography

A 73-year-old man, who is a former smoker, presented with worsening exertional dyspnea and a productive cough. The primary care physician heard fine crackles on chest auscultation, and a chest radiograph showed bilateral basal interstitial thickening. Pulmonary function tests demonstrated a moderate restriction and reduced diffusing capacity of the lungs for carbon monoxide. Idiopathic pulmonary fibrosis (IPF) was suspected. However, the high-resolution computed tomography scan of the chest revealed signs of lung fibrosis with a radiologic pattern indeterminate for usual interstitial pneumonia (UIP). Serological screening for autoimmune connective tissue diseases was negative. Bronchoalveolar lavage findings did not suggest any other differential diagnosis. A multidisciplinary team meeting proposed a lung biopsy during awake video-assisted thoracic surgery (VATS). The histologic findings were consistent with a UIP pattern and the patient started antifibrotic therapy with pirfenidone. This chapter describes the diagnostic and therapeutic process of an IPF with radiological findings indeterminate for UIP pattern. It discusses relevant topics such as Velcro crackles, multidisciplinary discussion, histopathologic criteria for UIP, and awake-VATS.