Idiopathic Pleuroparenchymal Fibroelastosis

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

• Rare disease with only 100+ cases reported in the literature to date²
• Affects both men and women equally¹
• Median age of onset: 57 years¹

• Lung, bone marrow, and hematopoietic cell transplantations^3,4
• Chemotherapy⁴
• Occupational exposures such as aluminosilicate dust⁵
• History of recurrent lower respiratory tract infections⁶
• Genetics/familial history⁷