Idiopathic pulmonary fibrosis natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

If left untreated, idiopathic pulmonary fibrosis will lead to respiratory failure and death. Common complications of idiopathic pulmonary fibrosis include pulmonary hypertension, lung cancer, and cardiovascular co-morbidity. The prognosis of idiopathic pulmonary fibrosis is usually poor with a survival rate of 2 to 5 years.

• If left untreated, idiopathic pulmonary fibrosis will lead to respiratory failure and death.
• The natural progression is different among the patients. Progression may occur in three different forms as the following:^[1]
  • Some patients may be stable and no significant worsening of respiratory functions be observed
  • Other patients’ lung functions get worse over years
  • Acute exacerbation of pulmonary fibrosis may be seen after disease stability

• Common complications of idiopathic pulmonary fibrosis include the following:^[2]
  • Pulmonary hypertension: Around 30% of IPF patients are complicated by pulmonary hypertension.
  • Lung cancer
  • Cardiovascular comorbidity
  • Obstructive sleep apnea
  • Combined pulmonary fibrosis and emphysema

• The prognosis of idiopathic pulmonary fibrosis is usually poor with a survival rate of 2 to 5 years.

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