Fast Five Quiz: Idiopathic Pulmonary Fibrosis Differential Diagnosis

Guy W. Soo Hoo, MD, MPH


November 20, 2019

In 2014, the US Food and Drug Administration approved the antifibrotic agents nintedanib and pirfenidone for the treatment of IPF. Although neither of these drugs is curative, both have been shown to slow the fibrotic changes associated with IPF, underscoring the importance of prompt, early diagnosis and treatment.

Nintedanib, a tyrosine kinase receptor blocker, preserves lung function by slowing fibrogenesis; it also may increase the length of time to a patient's first acute exacerbations of IPF. Pirfenidone, a pyridone derivative recommended for mild to moderate IPF, slows fibrogenesis by inhibiting collagen formation.

The 2018 updated clinical practice guidelines for IPF by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society include recommendations to facilitate early diagnosis and provide guidance on treatment.

Radiation therapy is not an appropriate treatment for IPF.

Support groups may be helpful, but this is not a reason for early diagnosis.

For more on antifibrotic agents for IPF, read here.

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