Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

Shioya, Makoto; Otsuka, Mitsuo; Yamada, Gen; Umeda, Yasuaki; Ikeda, Kimiyuki; Nishikiori, Hirotaka; Kuronuma, Koji; Chiba, Hirofumi; Takahashi, Hiroki

doi:10.1155/2018/6043053

Cited by 51 publications

(53 citation statements)

References 20 publications

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“…The higher incidence of pneumothorax or pneumomediastinum and lower BMI and FVC in the PPFE group were consistent with those in the previous reports . Previous studies for PPFE in patients with IPF or other ILD also support our findings . Oda et al ., in patients with IPF, reported that the histologically confirmed PPFE group showed lower BMI (18.6 vs 25.1), shorter anteroposterior diameter of the thorax (13.2 ± 1.4 vs 14.7 ± 1.2 cm), higher residual volume (1.8 vs 1.3 L) and PaCO 2 levels (44.6 vs 41.7 mm Hg) .…”

Section: Discussionsupporting

confidence: 93%

Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis

et al. 2020

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Background and objective PPFE is characterized by fibrosis in the pleura and subpleural lung parenchyma in the upper lobes, while other types of ILD, mainly UIP, can be observed in about half of the patients in their lower lobes. The aim of this study was to evaluate the clinical significance of the radiologically defined PPFE in patients with IPF. Methods Clinical data and chest CT images were retrospectively analysed in 445 patients with IPF (biopsy‐proven cases, n = 165). The radiological criteria of PPFE were defined as follows: (i) bilateral subpleural dense fibrosis with or without pleural thickening in the upper lobes, (ii) evidence of disease progression and (iii) no clinical evidence of identifiable aetiologies. Results The median follow‐up period was 43.0 months. The mean age of the patients was 66.4 years and 76.4% were male. PPFE was identified in 28 patients (6.3%). The PPFE group showed lower BMI and lung function (FVC and TLC) at baseline, more frequent pneumothorax and pneumomediastinum, higher decline rates in lung function and poorer prognosis during follow‐up than the no‐PPFE group. PPFE was an independent risk factor (HR = 2.953, 95% CI: 1.350–6.460, P = 0.007) for pneumothorax or pneumomediastinum, but not for mortality in patients with IPF. Conclusion Among patients with IPF, the PPFE group, when compared to the no‐PPFE group, showed lower BMI and lung function and showed more frequent complications and poorer survival during follow‐up.

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Section: Discussionsupporting

confidence: 93%

Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis

et al. 2020

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“…Our study showed that only FVC was a significant predictive factor for mortality in patients with PPFE. The results of a previous report support our findings: Shioya et al [30], in a study of 29 PPFE patients, showed that FVC (HR, 0.093; 95% CI, 0.083 to 0.982; p = 0.017) was an independent factor predicting mortality, along with sex, age, and physiology (GAP) index (HR, 2.510; 95% CI, 1.245 to 5.059; p = 0.010), in a multivariate Cox proportional hazard model. Previous studies have also reported other prognostic factors in PPFE patients [17,19,31].…”

Section: Discussionsupporting

confidence: 93%

Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up

Lee

Chae

Song

et al. 2021

Korean J Intern Med

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Background/Aims: Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE. Methods: A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed. Results: The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (100%). The 1-and 3-year survival rates were 90.2% and 84.5%, respectively. Conclusions: Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia.

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“…Recently, S hioya et al. [20] retrospectively analysed the characteristics of iPPFE versus IPF and found that the outcome of iPPFE was significantly worse than that of IPF. Consistent with these reports, the present study demonstrated a distinctly worse outcome of iPPFE diagnosed by MDD compared with other IIPs, including IPF.…”

Section: Discussionmentioning

confidence: 99%

Nationwide cloud-based integrated database of idiopathic interstitial pneumonias for multidisciplinary discussion

et al. 2019

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Multidisciplinary discussion (MDD) requiring close communication between specialists (clinicians, radiologists and pathologists) is the gold standard for the diagnosis of idiopathic interstitial pneumonias (IIPs). However, MDD by specialists is not always feasible because they are often separated by time and location. An online database would facilitate data sharing and MDD. Our aims were to develop a nationwide cloud-based integrated database containing clinical, radiological and pathological data of patients with IIPs along with a web-based MDD system, and to validate the diagnostic utility of web-based MDD in IIPs.Clinical data, high-resolution computed tomography images and lung biopsy slides from patients with IIPs were digitised and uploaded to separate servers to develop a cloud-based integrated database. Web-based MDD was performed using the database and video-conferencing to reach a diagnosis.Clinical, radiological and pathological data of 524 patients in 39 institutions were collected, uploaded and incorporated into the cloud-based integrated database. Subsequently, web-based MDDs with a pulmonologist, radiologist and pathologist using the database and video-conferencing were successfully performed for the 465 cases with adequate data. Overall, the web-based MDD changed the institutional diagnosis in 219 cases (47%). Notably, the MDD diagnosis yielded better prognostic separation among the IIPs than did the institutional diagnosis.This is the first study of developing a nationwide cloud-based integrated database containing clinical, radiological and pathological data for web-based MDD in patients with IIPs. The database and the web-based MDD system that we built made MDD more feasible in practice, potentially increasing accurate diagnosis of IIPs.

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Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

Cited by 51 publications

References 20 publications

Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis

Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis

Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up

Nationwide cloud-based integrated database of idiopathic interstitial pneumonias for multidisciplinary discussion

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