Distinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary Fibrosis

Oda, Toshiatsu; Ogura, Takashi; Kitamura, Hideya; Hagiwara, Eri; Baba, Tomohisa; Enomoto, Yasunori; Iwasawa, Tae; Okihara, Koji; Takemura, Tamiko; Sakai, Fumikazu; Hasegawa, Yoshinori

doi:10.1378/chest.13-2866

Cited by 127 publications

(153 citation statements)

References 19 publications

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“…Patients with a coexistent ILD other than fibroelastosis were observed in the present study as previously reported [5,[12][13][14]. The frequency of such cases was 75% in our report and might be higher than the report by REDDY et al [5] (43%); however, the patients in the other reports did not always accept SLB of the lower lobe.…”

supporting

confidence: 74%

“…The three IPPFE cases among these four cases did not have radiotherapy, chemotherapy or inhalational injuries, which are known aetiologies of intra-alveolar fibrosis with septal elastosis. Some reports have included PPFE cases without pleural thickening [4,5,8,12,13]. Further studies are required to clarify the significance of pleural thickening in the diagnosis of PPFE.…”

mentioning

confidence: 99%

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Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?

et al. 2015

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supporting

confidence: 74%

mentioning

confidence: 99%

Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?

et al. 2015

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“…Oda et al conducted a retrospective review of the medical records of 110 consecutive patients with IPF with a histologic UIP pattern, and reported that 11 of these patients met the radiologic criteria for PPFE, while nine patients fulfilled the histologic criteria of PPFE (16). Similarly, Piciucchi et al reported the case of IPPFE showing traction bronchiectasis and honeycombing by CT scan (7).…”

Section: Discussionmentioning

confidence: 99%

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

et al. 2016

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The patient was a 68-year-old man presenting with body weight loss and exertional dyspnea. Highresolution computed tomography of the chest showed dense subpleural consolidation with traction bronchiectasis and volume loss predominantly in bilateral apical lesions and upper lobes. A histopathological analysis of a specimen of the right upper lobe showed histological patterns which were consistent with idiopathic pleuroparenchymal fibroelastotis (IPPFE). Treatment with pirfenidone was introduced with the expectation of its potential benefit. The effect of pirfenidone was satisfactory, and a decline in forced vital capacity was inhibited during treatment. This is the first case report suggesting the efficacy of pirfenidone for patients with IPPFE.

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“…In problematic cases, 3 performance elastic fiber stain, such as elastic Van Gieson stain, would be useful because the stain demonstrates a more sparse, fragmented, and disorganized elastic fiber distribution in the areas of fibrosis in UIP 4 compared with more marked and established elastic fiber deposition in relation to the alveolar walls in PPFE, which is described as containing at least twice as much elastin compared with UIP. 8 Recently, lesion with combined patterns of UIP and PPFE has been noted; in such an instance Oda et al 11 proposed labeling the lesion as PPFE, and this entity with combined patterns is associated with a much more dismal outcome compared with either idiopathic pulmonary fibrosis or PPFE alone.…”

Section: Pathologic Differential Diagnosesmentioning

confidence: 99%

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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Distinct Characteristics of Pleuroparenchymal Fibroelastosis With Usual Interstitial Pneumonia Compared With Idiopathic Pulmonary Fibrosis

Cited by 127 publications

References 19 publications

Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?

Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?

A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone

Pleuroparenchymal Fibroelastosis of the Lung: A Review

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