Pulmonary Fibrosis: The Daily PANCE Blueprint

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Pulmonary Fibrosis: The Daily PANCE Blueprint

A 42-year-old female presents to the clinic with a chronic dry cough and dyspnea on exertion. Vitals are remarkable for SpO2 85% on room air. The patient denies fever, chills, and lower extremity swelling. On physical exam, you notice extensive clubbing and auscultate bibasilar inspiratory Velcro-like crackles. CT scan shows subpleural honeycombing and intralobular thickening with traction bronchiectasis. Which of the following is the most likely diagnosis?

A. Cryptogenic organizing pneumonia
B. Idiopathic pulmonary fibrosis
C. Protein alveolar proteinosis
D. Reactive airway syndrome
E. Sarcoidosis

Answer and topic summary

The answer is B. Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis is the most common type of idiopathic interstitial pneumonia. IPF specifically is an idiopathic form of chronic fibrosing interstitial pneumonia limited to just the lungs. It has a pattern of usual interstitial pneumonia (UIP) on high-resolution CT or on histology. Clinical features include non-productive cough, dyspnea on exertion, bibasilar crackles, and clubbing. A high-resolution CT scan should be obtained. Hallmarks include bilateral, peripheral and basilar predominant reticulonodular infiltrates, honeycombing with subpleural predominance, and intralobular thickening with traction bronchiectasis. Treatment includes oxygen, pulmonary rehab, medications (Ofev or Esbriet), and possibly lung transplant.

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Smarty PANCE Content Blueprint Review:

Covered under ⇒ PANCE Blueprint Pulmonary ⇒ Restrictive Pulmonary Disease (PEARLS) ⇒ Idiopathic pulmonary fibrosis

Also covered as part of the Internal Medicine Rotation topic lists