Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis

Oda, Toshiatsu; Sekine, Akimasa; Tabata, Erina; Iwasawa, Tae; Takemura, Tamiko; Ogura, Takashi

doi:10.3390/jcm10040846

Cited by 15 publications

(22 citation statements)

References 34 publications

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“…Oda et al . reported that the frequency and prognostic impact of radiological UIP patterns were different between IPPFE and secondary PPFE (mainly associated with HP or CTD) 30 . Khiroya et al .…”

Section: Discussionmentioning

confidence: 99%

Clinical, radiological and pathological features of idiopathic and secondary interstitial pneumonia with pleuroparenchymal fibroelastosis in patients undergoing lung transplantation

et al. 2022

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Aims Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, and pathological PPFE is also observed in patients with secondary interstitial pneumonia. This study aimed to evaluate the pathological findings associated with radiological PPFE‐like lesions and the clinical and morphological features of patients with pathological PPFE. Methods and results We retrospectively reviewed the pathology of the explanted lungs from 59 lung transplant recipients with radiological PPFE‐like lesions. Pathological PPFE lesions were identified in 14 patients with idiopathic disease and in 12 patients with secondary disease. Pathological PPFE was associated with previous pneumothorax, volume loss in the upper lobes, and a flattened chest. Patients with idiopathic disease and those with secondary disease with pathological PPFE had similar clinical, radiological and pathological findings, whereas fibroblastic foci were more common in patients with idiopathic disease, and patients with secondary disease more frequently showed alveolar septal thickening with elastosis or fibrosis. Post‐transplantation survival did not differ between patients with idiopathic and secondary disease with pathological PPFE (log‐rank; P = 0.57) and was similar between patients with idiopathic disease with pathological PPFE and those with idiopathic pulmonary fibrosis (IPF) (log‐rank; P = 0.62). Conclusions Not all patients with interstitial pneumonia with radiological PPFE‐like lesions have pathological PPFE. Characteristic clinical features can suggest the presence of pathological PPFE, and idiopathic and secondary cases with pathological PPFE are similar except for fibroblastic foci in idiopathic cases and alveolar septal thickening with elastosis or fibrosis in secondary cases. Patients with pathological PPFE have a similar prognosis to those with IPF after transplantation.

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Section: Discussionmentioning

confidence: 99%

Clinical, radiological and pathological features of idiopathic and secondary interstitial pneumonia with pleuroparenchymal fibroelastosis in patients undergoing lung transplantation

et al. 2022

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“…Recently, Oda et al . [ 13 ] conducted a study of 164 patients with PPFE, which has been the largest cohort to date, and showed that age, FVC, DLco, KL-6, and the complication of UIP were independent prognostic factors for patients with PPFE [ 13 ]. These studies suggested that low %FVC and the coexistence of UIP are strong predictors of survival in patients with PPFE.…”

Section: Discussionmentioning

confidence: 99%

“…Among them, serum KL-6 is the most sensitive biomarker in predicting the prognosis of IPF [ 39 , 40 ]. Some studies have shown that high serum KL-6 levels are associated with a poor prognosis in patients with PPFE [ 8 , 13 , 21 ]. Fibrotic lesions in patients with PPFE begin in the upper lobes and progress to the lower lobes [ 8 , 41 ].…”

Section: Discussionmentioning

confidence: 99%

“…Although the clinical course of PPFE shows considerable variation among patients, there are no established models for predicting the prognosis of PPFE. Previous studies have suggested several promising prognostic factors, including older age [ 13 ], male sex [ 9 , 12 , 14 , 15 ], dyspnea [ 12 ], pneumothorax events [ 16 ], lower body mass index (BMI) [ 17 ], coexistent ILD [ 7 , 8 , 10 , 15 , 18 ] or usual interstitial pneumonia (UIP) pattern in the lower lobes [ 8 , 13 , 18 – 20 ], lower elector spinae muscle attenuation on computed tomography (CT) [ 14 ], lower arterial blood oxygenation [ 17 ], lower forced vital capacity (FVC) [ 13 , 19 ], lower diffusing capacity of the lung for carbon monoxide (DL CO ) [ 13 ], and higher serum levels of Krebs von den Lungen-6 (KL-6) [ 8 , 13 , 21 ], and higher serum latent TGF-β binding protein-4 (LTBP-4) levels [ 22 ].…”

Section: Introductionmentioning

confidence: 99%

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A proposed prognostic prediction score for pleuroparenchymal fibroelastosis

et al. 2021

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Background Clinical course of pleuroparenchymal fibroelastosis (PPFE) shows considerable variation among patients, but there is no established prognostic prediction model for PPFE. Methods The prediction model was developed using retrospective data from two cohorts: our single-center cohort and a nationwide multicenter cohort involving 21 institutions. Cox regression analyses were used to identify prognostic factors. The total score was defined as the weighted sum of values for the selected variables. The performance of the prediction models was evaluated by Harrell’s concordance index (C-index). We also examined the usefulness of the gender-age-physiology (GAP) model for predicting the prognosis of PPFE patients. Results We examined 104 patients with PPFE (52 cases from each cohort). In a multivariate Cox analysis, a lower forced vital capacity (FVC [defined as FVC < 65%]; hazard ratio [HR], 2.23), a history of pneumothorax (HR, 3.27), the presence of a lower lobe interstitial lung disease (ILD) (HR, 2.31), and higher serum Krebs von den Lungen-6 (KL-6) levels (> 550 U/mL, HR, 2.56) were significantly associated with a poor prognosis. The total score was calculated as 1 × (FVC, < 65%) + 1 × (history of pneumothorax) + 1 × (presence of lower lobe ILD) + 1 × (KL-6, > 550 U/mL). PPFE patients were divided into three groups based on the prognostic score: stage I (0–1 points), stage II (2 points), and stage III (3–4 points). The survival rates were significantly different in each stage. The GAP stage was significantly associated with the prognosis of PPFE, but no difference was found between moderate (stage II) and severe (stage III) disease. Our new model for PPFE patients (PPFE Prognosis Score) showed better performance in the prediction of mortality in comparison to the GAP model (C-index of 0.713 vs. 0.649). Conclusions Our new model for PPFE patients could be useful for predicting their prognosis.

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“…The clinical course of the patient was reviewed by the attending physician, a respiratory specialist. Since no significant differences in the clinical course or outcome have been reported between idiopathic and secondary PPFE [ 14 ], this study does not include a detailed examination of the etiology of individual cases.…”

Section: Methodsmentioning

confidence: 99%

Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis

et al. 2022

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Background Pleuroparenchymal fibroelastosis (PPFE) is a rare fibrosing lung disease with a predilection for the upper lobe and its progression causes hypoventilation, resulting in hypercapnia. Even though the association between sleep-related hypoventilation (SRH) and chronic obstructive pulmonary disease was well documented, its impact in patients with PPFE was not evaluated. The aim of this study is to clarify the impact of SRH on prognosis in PPFE. Methods A retrospective review of the medical records of 52 patients with PPFE who underwent transcutaneous carbon dioxide monitoring during sleep was done. Patients were stratified into SRH (n = 28) and non-SRH (n = 24) groups based on American Academy of Sleep Medicine criteria. The impact of SRH on the prognosis of PPFE, as well as the clinical factors and comorbidities of PPFE associated with SRH, were evaluated. Results Forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and carbon monoxide diffusing capacity (DLco) in the SRH group were significantly lower than the non-SRH group (P < .01). Chronic pulmonary aspergillosis (CPA) was found at a higher rate in the SRH group (P = .02). The median survival time for SRH patients was 330 days, whereas roughly 80% of non-SRH patients were alive during the 3-year observation period (P < .01). Body mass index was a significant prognostic factor in PPFE patients with SRH (HR .78; 95% CI; .64–.94; P < .01). Home oxygen therapy (HOT) during the day and noninvasive positive pressure ventilation (NPPV) at night while sleeping tended to improve prognosis in the SRH group, as indicated by HR of .25 (P = .07). Conclusions SRH may be a poor prognostic factor for PPFE. Additionally, SRH may modify susceptibility to Aspergillosis in patients with PPFE. HOT plus NPPV may improve the disease outcomes in patients with SRH.

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Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis

Cited by 15 publications

References 34 publications

Clinical, radiological and pathological features of idiopathic and secondary interstitial pneumonia with pleuroparenchymal fibroelastosis in patients undergoing lung transplantation

Clinical, radiological and pathological features of idiopathic and secondary interstitial pneumonia with pleuroparenchymal fibroelastosis in patients undergoing lung transplantation

A proposed prognostic prediction score for pleuroparenchymal fibroelastosis

Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis

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