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Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?

Title: Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?
Authors: Perruzza, M; Fusha, E; Cameli, P; Capecchi, P L; SELVI, ELEONORA; Gentili, F; Mazzei, M A; Aversa, S; Spina, D; Di Lucia, D; Sestini, P; Luzzi, L; Bargagli, E
Contributors: Perruzza, M; Fusha, E; Cameli, P; Capecchi, P L; Selvi, Eleonora; Gentili, F; Mazzei, M A; Aversa, S; Spina, D; Di Lucia, D; Sestini, P; Luzzi, L; Bargagli, E
Publication Year: 2019
Collection: Università degli Studi di Siena: USiena air
Subject Terms: Diagnosi; Giant cell arteriti; Pleuroparenchymal Fibroelastosis; PPFE; Pleuroparenchymal fibroelastosi; high resolution computed tomography of the chest; HRCT; light scattering spectroscopy; LSS; magnetic resonance; MRI; positron emission tomography; PET
Description: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease characterized by the fibrotic thickening of subpleural and parenchymal areas of the upper lobes. It may be both idiopathic or secondary to infections, interstitial lung diseases and/or drug exposure. Often PPFE patients report recurrent lower respiratory tract infections, suggesting that repeated inflammatory alterations induced by pulmonary infections may contribute to the development/progression of PPFE. Here, we report for the first time the case of a patient affected by Giant cell Arteritis with histologically proven PPFE. The lung involvement in GCA is rare and interstitial lung diseases are usually reported as an uncommon clinical manifestation of GCA. Our patient is probably the first case presenting PPFE associated with GCA and we wonder if this is a real associative disease or a coincidence perhaps, secondary to drug effects.
Document Type: article in journal/newspaper
File Description: ELETTRONICO
Language: English
Relation: info:eu-repo/semantics/altIdentifier/pmid/31024793; info:eu-repo/semantics/altIdentifier/wos/WOS:000477769000019; volume:27; firstpage:1; lastpage:3; numberofpages:3; journal:RESPIRATORY MEDICINE CASE REPORTS; http://hdl.handle.net/11365/1072290
DOI: 10.1016/j.rmcr.2019.100843
Availability: http://hdl.handle.net/11365/1072290; https://doi.org/10.1016/j.rmcr.2019.100843
Rights: info:eu-repo/semantics/openAccess
Accession Number: edsbas.5C778AA5
Database: BASE