Skill Checkup: A 63-Year-Old Man With Persistent Cough and New Shortness of Breath

Zab Mosenifar, MD

Disclosures

April 21, 2022

On the basis of the patient's history and presentation, he probably has idiopathic pulmonary fibrosis (IPF). IPF is one of several diffuse parenchymal lung diseases. This is a heterogeneous group of lung disorders, both idiopathic and potentially related to occupational, environmental, drug, or radiation exposure, or systemic illness such as collagen-vascular disease. Adult patients older than 60 with newly detected interstitial lung disease (ILD) of apparently unknown cause are suspected of having IPF if they have unexplained symptomatic or asymptomatic patterns of bilateral fibrosis on chest radiography or chest CT, and/or bibasilar inspiratory crackles like the patient in this case.

IPF predominantly affects men, who typically present with chronic cough and progressive respiratory distress of 6 months or longer in duration. Most patients have a history of smoking. It is rare for middle-aged adults to be diagnosed with IPF, but those with familial pulmonary fibrosis may be at risk. As with other cardiopulmonary conditions, the presentation is usually nonspecific and includes both respiratory and systemic manifestations (eg, shortness of breath, persistent cough, loss of appetite, weight loss). Often, men older than 60 report gradually worsening exertional dyspnea and cough without a precipitating event or exposure. In rare cases, patients will present with an acute exacerbation (ie, worsening of dyspnea over a few weeks with no apparent cause, newly developed ground-glass opacification on high-resolution CT [HRCT]) and a background of lower lobe fibrotic lung disease.

Risk factors for developing IPF include gastroesophageal reflux, chronic viral infections such as Epstein Barr virus, and hepatitis C. Common comorbid conditions are sleep apnea, emphysema, lung cancer, pulmonary hypertension, and coronary artery disease.

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