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A schematic overview of the disease progression hypothesis in IPPFE. The alveolar structure collapses, forming zonal elastotic lesions in the early phase of IPPFE. Consequently, intra-alveolar fibrosis with septal elastosis arises, progressing inward and forming a two-layered fibroelastosis pattern in IPPFE. Fine elastic fibres are sometimes observed in old collagen-filled alveoli with septal elastosis
