Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?
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 repo...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2019-01-01
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| Series: | Respiratory Medicine Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2213007119300826 |
| _version_ | 1818962020108599296 |
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| author | M. Perruzza E. Fusha P. Cameli P.L. Capecchi E. Selvi F. Gentili M.A. Mazzei S. Aversa D. Spina D. Di Lucia P. Sestini L. Luzzi E. Bargagli |
| author_facet | M. Perruzza E. Fusha P. Cameli P.L. Capecchi E. Selvi F. Gentili M.A. Mazzei S. Aversa D. Spina D. Di Lucia P. Sestini L. Luzzi E. Bargagli |
| author_sort | M. Perruzza |
| collection | DOAJ |
| 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. Keywords: Pleuroparenchymal fibroelastosis, Giant cell arteritis, Diagnosis |
| first_indexed | 2024-12-20T12:22:41Z |
| format | Article |
| id | doaj.art-43ec1a773d3b4a4083a2bca386531c56 |
| institution | Directory Open Access Journal |
| issn | 2213-0071 |
| language | English |
| last_indexed | 2024-12-20T12:22:41Z |
| publishDate | 2019-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Respiratory Medicine Case Reports |
| spelling | doaj.art-43ec1a773d3b4a4083a2bca386531c562022-12-21T19:40:56ZengElsevierRespiratory Medicine Case Reports2213-00712019-01-0127Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype?M. Perruzza0E. Fusha1P. Cameli2P.L. Capecchi3E. Selvi4F. Gentili5M.A. Mazzei6S. Aversa7D. Spina8D. Di Lucia9P. Sestini10L. Luzzi11E. Bargagli12Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyPathology Section, Department of Human Pathology and Oncology, Siena University Hospital, Siena, ItalyPathology Section, Department of Human Pathology and Oncology, Siena University Hospital, Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, ItalyThoracic Surgery Unit, Department of Cardiothoracic and Vascular Surgery, Siena University Hospital, ItalyDepartment of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale M. Bracci 16, 53100 Siena, Italy; Corresponding author. UOC Malattie Respiratorie e Trapianto di Polmone, Policlinico Le Scotte, Viale Bracci, 53100 Siena, Italy.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. Keywords: Pleuroparenchymal fibroelastosis, Giant cell arteritis, Diagnosishttp://www.sciencedirect.com/science/article/pii/S2213007119300826 |
| spellingShingle | M. Perruzza E. Fusha P. Cameli P.L. Capecchi E. Selvi F. Gentili M.A. Mazzei S. Aversa D. Spina D. Di Lucia P. Sestini L. Luzzi E. Bargagli Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? Respiratory Medicine Case Reports |
| title | Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? |
| title_full | Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? |
| title_fullStr | Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? |
| title_full_unstemmed | Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? |
| title_short | Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: A coincidence or a novel phenotype? |
| title_sort | pleuroparenchymal fibroelastosis ppfe associated with giant cell arteritis a coincidence or a novel phenotype |
| url | http://www.sciencedirect.com/science/article/pii/S2213007119300826 |
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