Idiopathic Pleuroparenchymal Fibroelastosis
OVERVIEW
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare and relatively new condition that consists of fibrosis of the pleura and subpleural lung parenchyma, predominantly in the upper lobes.1
- 40% die from PPFE1
- 60% experience disease progression1
EPIDEMIOLOGY
- Rare disease with only 100+ cases reported in the literature to date2
- Affects both men and women equally1
- Median age of onset: 57 years1
RISK FACTORS
- Lung, bone marrow, and hematopoietic cell transplantations3,4
- Chemotherapy4
- Occupational exposures such as aluminosilicate dust5
- History of recurrent lower respiratory tract infections6
- Genetics/familial history7