Idiopathic pulmonary fibrosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Overview
If left untreated, idiopathic pulmonary fibrosis will lead to complete respiratory failure and death. Common complications of idiopathic pulmonary fibrosis include pulmonary hypertension, lung cancer, and cardiovascular comorbidities. The prognosis of idiopathic pulmonary fibrosis is usually poor with a survival rate from 2 to 5 years.
Natural History
- If left untreated, idiopathic pulmonary fibrosis will lead to complete 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
Complications
- Common complications of idiopathic pulmonary fibrosis include the following:
- Pulmonary hypertension
- Lung cancer
- Cardiovascular comorbidities
Prognosis
- The prognosis of idiopathic pulmonary fibrosis is usually poor with a survival rate from 2 to 5 years.
References
- ↑ Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK; et al. (2011). "An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management". Am J Respir Crit Care Med. 183 (6): 788–824. doi:10.1164/rccm.2009-040GL. PMC 5450933. PMID 21471066. Review in: Ann Intern Med. 2011 Jun 21;154(12):JC6-8