Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury

Rosenbaum, Jason N.; Butt, Yasmeen M.; Johnson, Karen A.; Meyer, Keith C.; Batra, Kiran; Kanne, Jeffrey P.; Torrealba, José

doi:10.1016/j.humpath.2014.10.007

Cited by 63 publications

(72 citation statements)

References 22 publications

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“…3,7 Although the idiopathic form of PPFE is included in the latest classification scheme of idiopathic interstitial pneumonia, 6 the changes may represent a form of chronic lung injury rather being a specific disease. 7 Its pathogenesis remains unclear, although it is proposed that the starting point may involve an element of acute lung injury or interstitial inflammation. 8 In fact, areas of diffuse alveolar damage in patients with restrictive allograft syndrome after lung transplantation with ensuing PPFE appear to support this hypothesis.…”

Section: Discussionmentioning

confidence: 99%

“…Subsequently, von der Thüsen 3 summarized the information gleaned from properly documented case series, which were mostly from the English literature. With that information as well as information from recent publications, 5,7,8 there have been about 120 cases reported in the world literature. The age range of the patients is from 13 to 87 years.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…6 However, although PPFE was initially thought to represent a specific entity and was originally believed to be rare, current literature suggests that PPFE may not be a specific entity and that it is likely to represent a form of chronic lung injury seen in association with a variety of clinicopathologic conditions. 5,7 In this review, we present the clinical, radiologic, and pathologic findings of patients with PPFE, including recently proposed diagnostic criteria. 7 The differential diagnoses on histology as well as the current controversy surrounding the entity of PPFE will also be discussed.…”

mentioning

confidence: 99%

“…5,7 In this review, we present the clinical, radiologic, and pathologic findings of patients with PPFE, including recently proposed diagnostic criteria. 7 The differential diagnoses on histology as well as the current controversy surrounding the entity of PPFE will also be discussed.…”

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confidence: 99%

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Pleuroparenchymal Fibroelastosis of the Lung: A Review

Cheng¹,

Chuah

2016

Archives of Pathology &Amp; Laboratory Medicine

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Described in Japan by Amitani et al in 1992, the entity of idiopathic upper lobe fibrosis was subsequently given the name pleuroparenchymal fibroelastosis (PPFE) in the English-speaking world. Pleuroparenchymal fibroelastosis is believed to be a rare disease characterized by a fibrosing process affecting the pleura and the subpleural lung parenchyma, with a predilection for the upper lobes. Uniquely, the fibrosing process is elastotic in nature, being associated with intra-alveolar fibrosis. The etiology of PPFE is unclear at this juncture, with many cases being considered as idiopathic forms of the disease. Conditions associated with PPFE include infections, bone marrow transplantation, and autoimmunity. In this review, we explore the clinical, radiologic, and pathologic features associated with PPFE in light of current understanding of the disease. Recent studies implicated that PPFE may not be as uncommon as claimed. The various differential diagnoses and implications of diagnosing PPFE are discussed.

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Section: Discussionmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

mentioning

confidence: 99%

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confidence: 99%

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Pleuroparenchymal Fibroelastosis of the Lung: A Review

Cheng¹,

Chuah

2016

Archives of Pathology &Amp; Laboratory Medicine

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“…Diagnosis of PPFE generally requires histological confirmation. Radiological features of the disease, generally detected by chest high-resolution computed tomography (HRCT), may include: pleural and subpleural fibrotic thickening with upper lobe predominance, traction bronchiectasis, architectural distortions, loss of volume, and reticulations [17][18][19][20][21][22]. In advanced stages, architectural distortion can involve adjacent and lower lobes.…”

mentioning

confidence: 99%

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

Bargagli¹,

Rottoli²,

Torricelli³

et al. 2018

Pathobiology

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Pleuroparenchymal fibroelastosis is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis. It was first described 25 years ago by Amitani et al. This report firstly describes a new variant or rare phenotype of PPFE with airway involvement, minimal pleuroparenchymal connections, and non-necrotizing granulomas.

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Morphological and molecular motifs of fibrosing pulmonary injury patterns

Jonigk

Stark

Braubach

et al. 2019

The Journal of Pathology CR

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Interstitial lung diseases encompass a large number of entities, which are characterised by a small number of partially overlapping fibrosing injury patterns, either alone or in combination. Thus, the presently applied morphological diagnostic criteria do not reliably discriminate different interstitial lung diseases. We therefore analysed critical regulatory pathways and signalling molecules involved in pulmonary remodelling with regard to their diagnostic suitability. Using laser-microdissection and microarray techniques, we examined the expression patterns of 45 tissue-remodelling associated target genes in remodelled and non-remodelled tissue samples from patients with idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP), non-specific interstitial pneumonia (NSIP), organising pneumonia (OP) and alveolar fibroelastosis (AFE), as well as controls (81 patients in total). We found a shared usage of pivotal pathways in AFE, NSIP, OP and UIP, but also individual molecular traits, which set the fibrosing injury patterns apart from each other and correlate well with their specific morphological aspects. Comparison of the aberrant gene expression patterns demonstrated that (1) molecular profiling in fibrosing lung diseases is feasible, (2) pulmonary injury patterns can be discriminated with very high confidence on a molecular level (86-100% specificity) using individual gene subsets and (3) these findings can be adapted as suitable diagnostic adjuncts.No conflicts of interest were declared.

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Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury

Cited by 63 publications

References 22 publications

Pleuroparenchymal Fibroelastosis of the Lung: A Review

Pleuroparenchymal Fibroelastosis of the Lung: A Review

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

Morphological and molecular motifs of fibrosing pulmonary injury patterns

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