ATS Pocket Guide 2013 - (Page 16)

IPF  DI AGNOSIS ▶Acute Exacerbation of IPF1 ■ Acute respiratory worsening occurs in about 5–10% of patients with IPF. ■ Acute exacerbation of IPF may be a manifestation of an unidentified respiratory complication (e.g., pulmonary emboli, infection) or may represent an inherent acceleration in the pathobiological processes involved in IPF. ■ Recent data from gene expression profiling of patients with acute exacerbation of IPF do not suggest an infectious etiology. ■ Historically, criteria for acute exacerbation of IPF have included the following: » Unexplained worsening of dyspnea within 1 month » Evidence of hypoxemia (defined by worsened or severely impaired gas exchange) » New radiographic alveolar infiltrates » Absence of an alternative explanation (e.g., infection, pulmonary embolism, pneumothorax, or heart failure) ■ Acute exacerbation can occur at any point in the course of IPF and occasionally can be its presenting manifestation. ■ Worsened cough, fever, and/or increased sputum have been observed. ■ There are no known risk factors for acute exacerbation of IPF. » There have been reports of acute respiratory decompensation after thoracic surgery and BAL. » It is unclear whether these events represent true acute exacerbations or complications of the respective procedures. ■ Acute exacerbation of IPF histologically manifests as acute or organizing diffuse alveolar damage, or, less commonly, organizing pneumonia in zones of relatively preserved lung tissue away from the most fibrotic regions. ■ Anecdotal experience indicates that sampling issues in some patients may result in specimens demonstrating only uncomplicated UIP or the organizing phase of diffuse alveolar damage without histologic evidence of underlying UIP. ▶Vital Statistics1 ■ Deaths from pulmonary fibrosis increase with increasing age. ■ Evidence suggests that mortality from pulmonary fibrosis has increased over the past 2 decades. ■ Using the most rigorous definition of IPF, the mortality rate in the United States in 2003 was 61.2 deaths per 1,000,000 in men and 54.5 per 1,000,000 in women. ■ In Japan, the mortality rate for IPF was estimated to be 33 per 1,000,000 in men and 24 per 1,000,000 in women. ■ The mortality burden attributable to IPF is higher than that of some cancers. ■ Recent evidence suggests that mortality from IPF in the United States is greater in the winter months. ■ Progressive lung disease is responsible for 60% of IPF deaths. ■ Additional causes of IPF-related morbidity and mortality include coronary artery disease, pulmonary embolism, and lung cancer. 16 Guidelines for the Diagnosis and Management of Idiopathic Pulmonary Fibrosis 

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ATS Pocket Guide 2013

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