Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis
Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics...
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| Format: | Article |
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MDPI AG
2021-02-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/10/4/846 |
| _version_ | 1797396088547704832 |
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| author | Tsuneyuki Oda Akimasa Sekine Erina Tabata Tae Iwasawa Tamiko Takemura Takashi Ogura |
| author_facet | Tsuneyuki Oda Akimasa Sekine Erina Tabata Tae Iwasawa Tamiko Takemura Takashi Ogura |
| author_sort | Tsuneyuki Oda |
| collection | DOAJ |
| description | Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary and idiopathic PPFE. Methods: We retrospectively reviewed the medical records of consecutive PPFE patients between January 1999 and December 2018. We identified 132 idiopathic PPFE patients and 32 secondary PPFE patients. Results: The incidence of interstitial lung disease (ILD) pattern different from the usual interstitial pneumonia (UIP) pattern in the lower lobes was higher in secondary PPFE patients (38.5%) than in idiopathic PPFE patients (61.5%, <i>p</i> = 0.02). The idiopathic and secondary PPFE groups did not differ significantly in terms of laboratory data, respiratory complications, and survival (median: 5.0 years vs. 4.1 years, <i>p</i> = 0.95). The presence of UIP pattern was independently associated with increased mortality in multivariate analyses in idiopathic PPFE patients, but not in secondary PPFE patients. Conclusions: The frequency and prognostic impact of UIP-pattern ILD differed between idiopathic and secondary PPFE patients. Lung transplantation should be considered in secondary PPFE patients with low diffusing capacity of the lungs for carbon monoxide (DLCO) regardless of lower-lobe ILD pattern. |
| first_indexed | 2024-03-09T00:46:13Z |
| format | Article |
| id | doaj.art-fb340eb4238348bd8f0ffe56eaa29ac2 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T00:46:13Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-fb340eb4238348bd8f0ffe56eaa29ac22023-12-11T17:33:46ZengMDPI AGJournal of Clinical Medicine2077-03832021-02-0110484610.3390/jcm10040846Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal FibroelastosisTsuneyuki Oda0Akimasa Sekine1Erina Tabata2Tae Iwasawa3Tamiko Takemura4Takashi Ogura5Kanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Yokohama 236-8651, JapanKanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Yokohama 236-8651, JapanKanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Yokohama 236-8651, JapanKanagawa Cardiovascular and Respiratory Center, Department of Radiology, Yokohama 236-8651, JapanKanagawa Cardiovascular and Respiratory Center, Department of Radiology, Yokohama 236-8651, JapanKanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Yokohama 236-8651, JapanBackground: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary and idiopathic PPFE. Methods: We retrospectively reviewed the medical records of consecutive PPFE patients between January 1999 and December 2018. We identified 132 idiopathic PPFE patients and 32 secondary PPFE patients. Results: The incidence of interstitial lung disease (ILD) pattern different from the usual interstitial pneumonia (UIP) pattern in the lower lobes was higher in secondary PPFE patients (38.5%) than in idiopathic PPFE patients (61.5%, <i>p</i> = 0.02). The idiopathic and secondary PPFE groups did not differ significantly in terms of laboratory data, respiratory complications, and survival (median: 5.0 years vs. 4.1 years, <i>p</i> = 0.95). The presence of UIP pattern was independently associated with increased mortality in multivariate analyses in idiopathic PPFE patients, but not in secondary PPFE patients. Conclusions: The frequency and prognostic impact of UIP-pattern ILD differed between idiopathic and secondary PPFE patients. Lung transplantation should be considered in secondary PPFE patients with low diffusing capacity of the lungs for carbon monoxide (DLCO) regardless of lower-lobe ILD pattern.https://www.mdpi.com/2077-0383/10/4/846pleuroparenchymal fibroelastosisUIPhypersensitivity pneumonitisinterstitial lung diseases |
| spellingShingle | Tsuneyuki Oda Akimasa Sekine Erina Tabata Tae Iwasawa Tamiko Takemura Takashi Ogura Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis Journal of Clinical Medicine pleuroparenchymal fibroelastosis UIP hypersensitivity pneumonitis interstitial lung diseases |
| title | Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis |
| title_full | Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis |
| title_fullStr | Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis |
| title_full_unstemmed | Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis |
| title_short | Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis |
| title_sort | comparison of clinical characteristics and outcomes between idiopathic and secondary pleuroparenchymal fibroelastosis |
| topic | pleuroparenchymal fibroelastosis UIP hypersensitivity pneumonitis interstitial lung diseases |
| url | https://www.mdpi.com/2077-0383/10/4/846 |
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