Fast Five Quiz: Interstitial Nonidiopathic Pulmonary Fibrosis Management

Setu K. Patolia, MD, MPH


February 12, 2021

Risk factors of acute exacerbations of IPF include low forced vital capacity, gastroesophageal reflux disease, and greater area of disease on high-resolution CT scan.

An international working group defined an acute exacerbation as a clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormality. According to this definition, the deterioration does not necessarily have to be idiopathic, considering that acute respiratory decline due to a known cause like infection appears to have similar clinical features and prognosis to idiopathic decline. Similarly, a consensus statement published in 2007 defined acute exacerbations of IPF as being of unknown etiology and associated with new high-resolution CT scan findings and progressive dyspnea.

Mechanical ventilation support is currently not recommended for the majority of patients with respiratory failure due to IPF. However, data suggest that mechanical ventilation support might be associated with lower mortality rates than previously reported, and a subgroup of patients might even benefit, particularly from the noninvasive ventilation variety.

To date, no treatments for acute exacerbations have been proven, but patients are usually hospitalized and receive supplemental oxygen and broad-spectrum antibiotic therapy. International treatment guidelines offer a weak recommendation for high-dose corticosteroids, but supportive care remains the mainstay of treatment.

Learn more about imaging studies in DPLDs.


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