Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection?

Hirota, Takako; Fujita, Masaki; Matsumoto, Takemasa; Higuchi, Terumi; Shiraishi, Takeshi; Minami, Masato; Okumura, Meinoshin; Nabeshima, Kazuki; Watanabe, Kentaro

doi:10.1183/09031936.00103912

Cited by 51 publications

(30 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Some patients possess a familial history [2,6], medical history of chemotherapy [2] or recurrent infection [6], lung transplantation [17], or are positive for autoantibodies [6]. Furthermore, overexpression of transforming growth factor-α (TGF-α) may induce progressive interstitial and pleural fibrosis with body weight loss [18,19], and these changes are independent of TGF-β [19].…”

Section: Discussionmentioning

confidence: 99%

Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF)

et al. 2014

View full text Add to dashboard Cite

BackgroundThe pathological appearance of idiopathic pleuroparenchymal fibroelastosis (IPPFE) with hematoxylin-eosin staining is similar to that of usual interstitial pneumonia (UIP) in patients with idiopathic pulmonary fibrosis (IPF). The amount of elastic fibers (EF) and detailed differences between IPPFE and IPF have not been fully elucidated. The aim of this study was to quantify the EF and identify the differences between IPPFE and IPF.MethodsWe evaluated six patients with IPPFE and 28 patients with IPF who underwent surgical lung biopsy or autopsy. The patients’ clinical history, physical findings, chest high-resolution computed tomography (HRCT) findings, and pathological features of lung specimens were retrospectively evaluated. The amounts of EF in lung specimens were quantified with Weigert’s staining using a camera with a charge-coupled device and analytic software in both groups.ResultsFewer patients with IPPFE than IPF had fine crackles (50.0% vs. 96.4%, p = 0.012). Patients with IPPFE had a lower forced vital capacity (62.7 ± 10.9% vs. 88.6 ± 21.9% predicted, p = 0.009), higher consolidation scores on HRCT (1.7 ± 0.8 vs. 0.3 ± 0.5, p < 0.0001), lower body mass indices (17.9 ± 0.9 vs. 24.3 ± 2.8, p < 0.0001), and more pneumothoraces than did patients with IPF (66.7 vs. 3.6%, p = 0.002). Lung specimens from patients with IPPFE had more than twice the amount of EF than did those from patients with IPF (28.5 ± 3.3% vs. 12.1 ± 4.4%, p < 0.0001). The amount of EF in the lower lobes was significantly lower than that in the upper lobes, even in the same patient with IPPFE (23.6 ± 2.4% vs. 32.4 ± 5.5%, p = 0.048). However, the amount of EF in the lower lobes of patients with IPPFE was still higher than that of patients with IPF (23.6 ± 2.4% vs. 12.2 ± 4.4%, p < 0.0001).ConclusionMore than twice the amount of EF was found in patients with IPPFE than in those with IPF. Even in the lower lobes, the amount of EF was higher in patients with IPPFE than in those with IPF, although the distribution of lung EF was heterogeneous in IPPFE specimens.

show abstract

Section: Discussionmentioning

confidence: 99%

Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF)

et al. 2014

View full text Add to dashboard Cite

show abstract

“…In addition, they noted the presence of diffuse parenchymal fibroelastosis involving both upper and lower lobes of the lung in all of their cases, suggesting that this disease may be more diffuse than once thought. As such, they opined that PPFE may represent a pattern of chronic lung injury rather than a specific entity and that this form of chronic injury can coexist with a variety of clinicoradiologic conditions, a hypothesis similar to that raised by Hirota et al 14 Hence, they proposed a set of diagnostic criteria for a diagnosis of PPFE, the criteria comprising the presence of multilobar subpleural and/or centrilobular fibrous interstitial pneumonia characterized by more than 80% proliferation of elastic fibers in the nonatelectatic lung in association with absent to scant chronic inflammation and no or rare granulomas.…”

Section: Pathologic Differential Diagnosesmentioning

confidence: 96%

Pleuroparenchymal Fibroelastosis of the Lung: A Review

Cheng¹,

Chuah

2016

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

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.

show abstract

“…The main concern of the large-scale studies performed was that they did not assess the effect of roflumilast in addition to a combination product such as long-acting b 2 -agonists (LABA) or long-acting muscarinic agents (LAMA) combined with inhaled corticosteroids (ICS) [2]. A novel technology called functional respiratory imaging (FRI) defines, in great detail, lung geometry and regional changes in airway volume and resistance [3][4][5]. By assessing changes close to the site of action of the intervention, the method is more sensitive (higher effect size) compared with standard pulmonary function tests (PFT) [3].…”

Section: To the Editormentioning

confidence: 99%

“…A few cases of PPFE have occurred amongst siblings [1,3]. PPFE has also been reported to be associated with a history of stem cell transplantation [4,5] or as a manifestation of chronic lung rejection in lung transplant recipients [5]. Recently, REDDY et al [6] evaluated the pathologic and imaging results in 12 patients who fulfilled the established criteria for idiopathic PPFE.…”

mentioning

confidence: 99%