Idiopathic pulmonary fibrosis

Background

• Median survival time 3 yrs after diagnosis
• Prevalence about 10-20 cases per 100,000 people
• Acute exacerbations carry mortality up to 80%
• 60% die from idiopathic pulmonary fibrosis, others die from:
  • VTE
  • Cardiovascular (ACS, CHF)
  • Infection

DDx

Pulmonary Fibrosis

• Interstitial pneumonias (acute, lymphocytic)
• Lung malignancy
• Aspiration pneumonia or pneumonitis
• Bacterial, viral, or fungal pneumonia
• Cryptogenic organizing pneumonia
• Interstitial lung disease associated with collagen vascular disease
• Drug-induced pulmonary toxicity (amiodarone, bleomycin, amphotericin B, carbamazepine, etc.)
• Eosinophilic granuloma (Histiocytosis X)
• Radiation pneumonitis
• Sarcoidosis
• Pneumoconiosis (Workplace exposure)
  • Asbestosis
  • Berylliosis
  • Chemical worker's lung
  • Coal worker's pneumoconiosis
  • Silicosis

See Also

• Pulmonary hypertension

Sources

• Collard HR et al. Acute Exacerbations of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2007 Oct 1; 176(7): 636–643.
• Ryerson CJ et al. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J. 2015 Aug;46(2):512-20.
• Juarez MM et al. Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies. J Thorac Dis. 2015 Mar; 7(3): 499–519.