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: Ahmed Elsaiey, MBBCH [2]
Overview
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.
Natural History
- 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
Complications
- 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
Prognosis
- The prognosis of idiopathic pulmonary fibrosis is usually poor with a survival rate of 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
- ↑ Raghu G, Amatto VC, Behr J, Stowasser S (2015). "Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review". Eur Respir J. 46 (4): 1113–30. doi:10.1183/13993003.02316-2014. PMID 26424523.
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