Significant increases in the density and number of lymphatic vessels in pleuroparenchymal fibroelastosis

Kinoshita, Yoshiaki; Watanabe, Kentaro; Ishii, Hiroshi; Kushima, Hisako; Fujita, Masaki; Nabeshima, Kazuki

doi:10.1111/his.13634

Cited by 14 publications

(8 citation statements)

References 48 publications

(72 reference statements)

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“…Watanabe et al [13] suggested that the diagnostic criteria of IPPFE should first include its clinical characteristics and then evolve into more refined and simplified criteria, as has happened with the diagnostic criteria of IPF [28,29]. One of the important clinical challenges for the diagnostic criteria of IPPFE is differentiating IPPFE patients from IPF patients [12,13,24,26]. Physiological criteria of IPPFE are essential because they can distinguish the two entities with high sensitivity and specificity [13].…”

Section: Discussionmentioning

confidence: 99%

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Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis

Ikeda

Kinoshita

Ueda

et al. 2020

JCM

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Background: Diagnostic criteria of idiopathic pleuroparenchymal fibroelastosis (IPPFE) were recently proposed, including physiological criteria of the body mass index (BMI) and percentage of the predicted values of residual volume (RV)/total lung capacity (TLC) (RV/TLC %pred.). The aim of this study was to evaluate (i) whether the physiologic criteria are useful for the diagnosis and (ii) whether the flat chest index, defined as the ratio of the anteroposterior diameter to the transverse diameter of the thoracic cage, could be an alternative parameter to RV/TLC %pred. Methods: We selected consecutive IPPFE patients and idiopathic pulmonary fibrosis (IPF) patients. We examined the diagnostic sensitivity and specificity of the physiological criteria and flat chest index for differentiating IPPFE patients from IPF patients. Results: This study included 37 IPPFE patients and 89 IPF patients. The physiological criteria distinguished IPPFE patients from IPF patients with a sensitivity of 78.6% and specificity of 88.0%. The combination of the flat chest index and BMI was also effective for differentiation (sensitivity of 82.1% and specificity of 89.3%). Conclusion: We verified the good performance of the physiologic criteria in a different cohort. When the RV/TLC is not measured, using the flat chest index instead of RV/TLC %pred. may be reasonable.

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Section: Discussionmentioning

confidence: 99%

“…The respiratory function parameters examined were the FVC, FEV 1 , functional residual capacity (FRC), RV, TLC, and diffusion capacity of the lung for carbon monoxide (DLco). The respiratory function parameters were measured as previously described [24][25][26].…”

Section: Clinical Characteristics and Respiratory Function Datamentioning

confidence: 99%

Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis

Ikeda

Kinoshita

Ueda

et al. 2020

JCM

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“…The PPFE-like lesions appeared to develop when peripheral consolidations due to sarcoidosis were limited to the apex regions of the upper lobes, suggesting possible similarities in the predominance and pathology of lesion formation between PPFE and chronic sarcoidosis. One of the pathological characteristics of PPFE is lymphatic proliferation, which may be a compensatory event secondary to impaired lymphatic drainage (13). It is necessary to investigate whether or not subpleural consolidations on chest CT in the present case were associated with particular pathological findings, such as fibrosis and the confluence of granulomas; increases in elastic fibers, proliferation of podoplaninpositive myofibroblasts (14), and lymphatic proliferation (13) as seen in PPFE cases; and increases in smooth muscle and collagenous fibers as seen in idiopathic pulmonary fibrosis/usual interstitial pneumonia cases.…”

Section: Discussionmentioning

confidence: 99%

Paradoxical Formation of a Pleuroparenchymal Fibroelastosis-like Lesion in the Chronic Course of Pulmonary Sarcoidosis

et al. 2022

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We herein report the long-term changes in chest computed tomography (CT) findings from early sarcoidosis lesions to pleuroparenchymal fibroelastosis (PPFE)-like lesions in a 30-year-old man with granulomas on a transbronchial lung biopsy. Multiple bilateral micronodular and nodular opacities around the bronchovascular bundle in the upper lobes detected by chest CT in 2004 disappeared, but paradoxically, peripheral consolidations continued to grow at the periphery of the original lesions. Chest CT in 2017 confirmed the progression of bilateral shrinkage of the upper lobe, spread of peripheral consolidations and wedge-shaped opacities below the first rib, and bronchiectatic air bronchograms, confirming PPFE-like lesions.

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“…Histologically, PPFE is characterized by 1) intense fibrosis of the visceral pleura; 2) prominent, homogenous, subpleural fibroelastosis; 3) sparing of the parenchyma distant from the pleura; 4) mild, patchy lymphoplasmacytic infiltrates; and 5) small numbers of fibroblastic foci ( 8 ). Kinoshita et al ( 7 ) reported that the number of lymphatic vessels in PPFE was significantly higher than in the normal lungs, apical cap, and idiopathic pulmonary fibrosis. They also reported that an increased lymphatic vessel density was correlated with the characteristic physiology of PPFE, such as a flattened chest cage and high RV/TLC ratio.…”

Section: Discussionmentioning

confidence: 99%

“…No granulomas were observed. Many D2-40-positive lymphatic vessels were seen within the elastofibrotic lesions (approximate lymph vessel density was 5.5%) ( 7 ) ( Fig. 6B ).…”

Section: Case Reportmentioning

confidence: 99%

An Autopsy Case of Idiopathic Pleuroparenchymal Fibroelastosis with Left Vocal Cord Paralysis and a Rapid Deterioration without an Acute Exacerbation

et al. 2020

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Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a generally slow-progressing rare disorder of unknown etiology. The direct cause of death in cases of IPPFE is rarely investigated. We experienced an autopsy case of a Japanese man with IPPFE and found aspiration pneumonia to be the major trigger of death. The individual had left vocal cord paralysis at admission, which may have contributed to aspiration pneumonia, and which probably was affected by the fibrous adhesion of the left apex of the chest wall resulting from IPPFE. The prevention of aspiration pneumonia is important for maintaining the respiratory function, especially in IPPFE patients with repeated pneumothorax.

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Significant increases in the density and number of lymphatic vessels in pleuroparenchymal fibroelastosis

Abstract: Significant increase in the density and number of lymphatic vessels is a supportive characteristic that enables the differentiation of PPFE from IPF and apical cap.

Cited by 14 publications

References 48 publications

Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis

Physiological Criteria Are Useful for the Diagnosis of Idiopathic Pleuroparenchymal Fibroelastosis

Paradoxical Formation of a Pleuroparenchymal Fibroelastosis-like Lesion in the Chronic Course of Pulmonary Sarcoidosis

An Autopsy Case of Idiopathic Pleuroparenchymal Fibroelastosis with Left Vocal Cord Paralysis and a Rapid Deterioration without an Acute Exacerbation

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