Pleuroparenchymal fibroelastosis is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis. It was first described 25 years ago by Amitani et al. This report firstly describes a new variant or rare phenotype of PPFE with airway involvement, minimal pleuroparenchymal connections, and non-necrotizing granulomas.

Bargagli, E., Rottoli, P., Torricelli, E., Allegrini, C., Dubini, A., Bennett, D., et al. (2018). Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity. PATHOBIOLOGY, 85(5-6), 276-279 [10.1159/000492431].

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

Bargagli, Elena;Rottoli, Paola;Bennett, David;Luzzi, Luca;
2018-01-01

Abstract

Pleuroparenchymal fibroelastosis is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis. It was first described 25 years ago by Amitani et al. This report firstly describes a new variant or rare phenotype of PPFE with airway involvement, minimal pleuroparenchymal connections, and non-necrotizing granulomas.
Bargagli, E., Rottoli, P., Torricelli, E., Allegrini, C., Dubini, A., Bennett, D., et al. (2018). Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity. PATHOBIOLOGY, 85(5-6), 276-279 [10.1159/000492431].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11365/1062456