Idiopathic pulmonary fibrosis
Other Names for this Disease
- Familial idiopathic pulmonary fibrosis
- Fibrocystic pulmonary dysplasia
- Fibrosing alveolitis
- Fibrosing alveolitis, cryptogenic
- Hamman-Rich disease
- older age at diagnosis
- extensive cigarette smoking
- lower body mass index (BMI)
- more severe physiologic impairment
- greater extent of disease on imaging studies
- the development of other complications or conditions (eg, pulmonary hypertension, emphysema, and bronchogenic cancer)
The natural history of IPF is not completely understood. While it usually follows a course of progressive deterioration, some people remain stable for extended periods and individual outcomes can vary significantly. Still, long-term survival of IPF is not expected.
It now appears that three potential disease courses exist, but there is not currently a definitive way to predict the course in an affected person. They are:
a) slowly progressive disease (the most common);
b) disease marked by episodes of acute exacerbations; and
c) rapidly progressive disease.
- Talmadge E King, Jr. Treatment of idiopathic pulmonary fibrosis. UpToDate. Waltham, MA: UpToDate; July, 2014; Accessed 7/31/2014.
- Eric B Meltzer and Paul W Noble. Idiopathic pulmonary fibrosis. Orphanet Journal of Rare Diseases. 2008; 3:8:http://www.ojrd.com/content/3/1/8. Accessed 7/30/2014.