Pleuroparenchymal fibroelastosis
Other Names: Idiopathic pleuroparenchymal fibroelastosis; IPPFE; Idiopathic pleuropulmonary fibroelastosis
Pleuroparenchymal fibroelastosis (PPFE) is a newly described form of interstitial lung disease that originates in the upper lung zones and typically progresses to involve the entire lung. The disease may be idiopathic but is often associated with other pre- or coexisting conditions. Pneumothorax is a common complication and can occur at presentation or at other times during the course of the disease.
NIH genetic and rare disease info[edit source]
Pleuroparenchymal fibroelastosis is a rare disease.
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