Idiopathic pulmonary fibrosis (IPF) is a debilitating and almost uniformly fatal disease. IPF is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP).1 The definition of IPF requires the exclusion of other forms of interstitial pneumonia including other idiopathic interstitial pneumonias and ILD associated with environmental exposure, medication, or systemic disease.

Symptoms include exertional dyspnea and a dry cough that gradually worsen. Most patients are afflicted with progressive difficulty breathing and shortness of breath. Some patients progress gradually while others appear to experience periods of relative stability interrupted by unpredictable acute exacerbations.2 The median survival time from diagnosis of IPF is three to five years.3

Nintedanib and pirfenidone were approved by the FDA in October 2014 for the treatment of patients with IPF. In clinical studies these treatments slow the rate of decline of forced vital capacity (FVC) by approximately 50% but do not cure the disease. Drug combinations and new therapies are under investigation.4

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References

  1. Raghu G, Collard HR, Egan JJ, et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
  2. Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis. Clin Chest Med. 2012;33(1):59-68.
  3. Olson AL, Swigris JJ. Idiopathic pulmonary fibrosis: diagnosis and epidemiology. Clin Chest Med. 2012;33(1):41-50.
  4. Raghu G, Rochwerg B, Zhang Y, et al; American Thoracic Society; European Respiratory society; Japanese Respiratory Society; Latin American Thoracic Association. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3-19.

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