Next Article in Journal
Is Urinary Netrin-1 a Good Marker of Tubular Damage in Preterm Newborns?
Next Article in Special Issue
Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis
Previous Article in Journal
Medio-Lateral and Flexion-Extension Gap Imbalances in Mechanically Aligned Total Knee Arthroplasty Using Measured Resection Technique in Korean Patients: 3D Simulation
Previous Article in Special Issue
Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis
Article

Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis

1
Kanagawa Cardiovascular and Respiratory Center, Department of Respiratory Medicine, Yokohama 236-8651, Japan
2
Kanagawa Cardiovascular and Respiratory Center, Department of Radiology, Yokohama 236-8651, Japan
*
Author to whom correspondence should be addressed.
Academic Editor: Hiroshi Ishii
J. Clin. Med. 2021, 10(4), 846; https://doi.org/10.3390/jcm10040846
Received: 20 January 2021 / Revised: 12 February 2021 / Accepted: 15 February 2021 / Published: 18 February 2021
(This article belongs to the Special Issue Pleuroparenchymal Fibroelastosis: Obstacles and Challenges)
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%, p = 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, p = 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. View Full-Text
Keywords: pleuroparenchymal fibroelastosis; UIP; hypersensitivity pneumonitis; interstitial lung diseases pleuroparenchymal fibroelastosis; UIP; hypersensitivity pneumonitis; interstitial lung diseases
Show Figures

Figure 1

MDPI and ACS Style

Oda, T.; Sekine, A.; Tabata, E.; Iwasawa, T.; Takemura, T.; Ogura, T. Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis. J. Clin. Med. 2021, 10, 846. https://doi.org/10.3390/jcm10040846

AMA Style

Oda T, Sekine A, Tabata E, Iwasawa T, Takemura T, Ogura T. Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis. Journal of Clinical Medicine. 2021; 10(4):846. https://doi.org/10.3390/jcm10040846

Chicago/Turabian Style

Oda, Tsuneyuki, Akimasa Sekine, Erina Tabata, Tae Iwasawa, Tamiko Takemura, and Takashi Ogura. 2021. "Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis" Journal of Clinical Medicine 10, no. 4: 846. https://doi.org/10.3390/jcm10040846

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Back to TopTop