Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study

Tanizawa, Kiminobu; Handa, Tomohiro; Kubo, Takeshi; Chen‐Yoshikawa, Toyofumi F.; Aoyama, Akihiro; Motoyama, Hideki; Hijiya, Kyoko; Yoshizawa, Akihiko; Oshima, Yasuhiro; Ikezoe, Kohei; Tokuda, Shinsaku; Nakatsuka, Yoshinari; Murase, Yuko; Nagai, Sonoko; Muro, Shigeo; Oga, Toru; Chin, Kazuo; Hirai, Toyohiro; Date, Hiroshi

doi:10.1186/s12931-018-0860-6

Cited by 38 publications

(39 citation statements)

References 27 publications

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“…The radiological criteria for PPFE, modified from Reddy et al . 's criteria and other articles, were defined as follows: (i) bilateral subpleural dense fibrosis with or without pleural thickening in the upper lobes, (ii) confirmation of disease progression (defined as an increase in the upper lobe consolidation with or without pleural thickening and/or a decrease in upper lobe volume on serial radiological assessment) and (iii) exclusion of other identifiable aetiologies (such as history of radiation therapy in the upper lung zones and active pulmonary infection) or HRCT findings such as multiple lung or pleural calcification and unilateral lesion. Patients were classified as definite PPFE cases if all criteria were met and as possible PPFE cases if criteria (i) and (iii) were met.…”

Section: Methodsmentioning

confidence: 99%

“…Idiopathic pulmonary fibrosis (IPF) is characterized by a UIP pattern on high‐resolution computed tomography (HRCT) or surgical lung biopsy and has a poor prognosis with frequent complications such as pneumothorax . There have been some reports on the clinical significance of PPFE in IPF patients . Oda et al ., in 110 patients with biopsy‐proven IPF, showed that those with histologically confirmed PPFE ( n = 9) had different clinical features than those without (IPF alone, n = 99); the PPFE group showed a significantly higher residual volume, higher partial pressure of carbon dioxide in arterial blood (PaCO 2 ), higher complication rate of pneumothorax and pneumomediastinum and numerically shorter survival time (31.5 vs 82.1 months, P = 0.13) than the no‐PPFE group.…”

Section: Introductionmentioning

confidence: 99%

“…Oda et al ., in 110 patients with biopsy‐proven IPF, showed that those with histologically confirmed PPFE ( n = 9) had different clinical features than those without (IPF alone, n = 99); the PPFE group showed a significantly higher residual volume, higher partial pressure of carbon dioxide in arterial blood (PaCO 2 ), higher complication rate of pneumothorax and pneumomediastinum and numerically shorter survival time (31.5 vs 82.1 months, P = 0.13) than the no‐PPFE group. Tanizawa et al ., in 118 fibrotic ILD patients registered for lung transplantation, reported that those with radiological PPFE ( n = 30) had lower body mass index (BMI) and forced vital capacity (FVC) as well as more frequent history of pneumothorax, higher diffusing capacity of the lung for carbon monoxide (DL CO ), less desaturation on the 6‐min walk test (6MWT) and hypercapnia, and marginally better survival (hazard ratio (HR) = 0.51, P = 0.07) than those without . Despite these reports, it is still unclear whether the presence of PPFE can have an impact on the clinical course and prognosis of patients with IPF due to the small number of subjects or limited patient selection.…”

Section: Introductionmentioning

confidence: 99%

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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: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis

et al. 2020

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“…Tanizawa et al [21] reported that, in 118 patients with fibrotic ILD who registered for lung transplantation, BMI was significantly different between the radiologically defined PPFE and no PPFE groups (15.9 kg/m² vs. 21.5 kg/m², p < 0.001). Oda et al [16], in 108 IPF patients, reported that the combined PPFE group showed lower BMI (18.6 ± 1.8 kg/m² vs. 25.1 ± 3.6 kg/m², p < 0.01) and higher RV/TLC (42.6% ± 8.7% vs. 33.1% ± 7.6%, p < 0.01) than the IPF only group.…”

Section: Discussionmentioning

confidence: 99%

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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“…Although it reported that the median survival time for CTD-ILD patients was roughly 6.5 years, and the mortality rate due to CTD attributable to ILD was approximately 123.6 per 1000 person years [2,3], but the progression and prognosis of CTD-ILD varies widely between individuals [4]. In general, the poor prognosis of ILD was reported to correlate with the dyspnea score, pulmonary function degree [5,6], patterns of high-resolution computed tomography (HRCT) scans [7,8]. Limited by the patient's cooperation, we try to find some reliable and less expensive serum biomarkers which are also easy to detect.…”

Section: Introductionmentioning

confidence: 99%

Vitamin D levels are prognostic factors for connective tissue disease associated interstitial lung disease (CTD-ILD)

Gao

Zhao

Qiu

et al. 2020

Aging

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Objective: Vitamin D deficiency was associated with CTD-ILD and reduced lung function. We sought to confirm that lower Vitamin D level would be related to shorter survival times. Results: The CTD-ILD patients had lower Vitamin D level (P<0.05). Among patients with CTD-ILD who have improved lung function after treatment, elevation of Vitamin D level was positively associated with ΔFVC (%), ΔFEV1(%) and ΔDLCO-SB (%). The median survival time of patients with high serum 25(OH)D level was significantly longer than the patients with low 25(OH)D level group (16.5 months vs14.0 months, P=0.007). The Vitamin D was identified as an independent prognostic factor with a hazard ratio of 0.869 (95% CI 0.772-0.977, P =0.019). Conclusions: Vitamin D level was lower in patients with CTD-ILD and associated with poor prognosis. Continuous levels of Vitamin D may be an important serum biomarker of prognosis. Methods: 85 CTD-ILD patients, 71 Idiopathic pulmonary fibrosis (IPF) patients and 78 healthy control patients were included in the study. In the subgroup analysis, the CTD-ILD patients were divided into anti-MDA5 antibody-positive group and anti-MDA5 antibody-negative group according to the serum autoantibodies results. The survival analysis evaluated effect of Vitamin D level on disease prognosis.

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Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study

Cited by 38 publications

References 27 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

Vitamin D levels are prognostic factors for connective tissue disease associated interstitial lung disease (CTD-ILD)

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