Pleuroparenchymal fibroelastosis

Cottin, Vincent; Diesler, Rémi; Bonniaud, Philippe; Valenzuela, Claudia

doi:10.1097/mcp.0000000000000907

Cited by 23 publications

(32 citation statements)

References 72 publications

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“…Further, the hypothesis that radiological PPFE is similar in pathogenesis to idiopathic/secondary PPFE can be considered. However, there is still insufficient evidence on pharmacological treatment for idiopathic/secondary PPFE, including antifibrotic agents such as nintedanib and pirfenidone as well as corticosteroids 36 37. To reduce pneumonia-related deaths, pneumonia prevention strategies,38 39 including pneumococcal and influenza vaccinations, should be considered.…”

Section: Discussionmentioning

confidence: 99%

Prognostic significance of radiological pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study

Aono

Hozumi

Kono

et al. 2022

Thorax

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BackgroundMycobacterium aviumcomplex (MAC) causes chronic respiratory infectious diseases with diverse clinical features and prognoses. Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterised by pleural fibrosis with subjacent intra-alveolar fibrosis and alveolar septal elastosis, with unique chest high-resolution CT (HRCT) features (radiological PPFE). An association between recurrent respiratory infections and PPFE formation has been hypothesised; however, the clinical significance of PPFE in MAC lung disease remains unclear.MethodsThis retrospective, multicentre study investigated the prevalence of radiological PPFE in patients with MAC lung disease and its association with clinical features and outcomes. Radiological PPFE was diagnosed on the basis of HRCT findings. Prognostic factors were identified using Cox proportional hazards and Fine-Gray models.ResultsOf 850 consecutive patients with definite MAC lung disease, 101 (11.9%) exhibited radiological PPFE. Patients with radiological PPFE had unique characteristics, such as lower body mass index, lower survival rate (5-year cumulative survival rate, 63.1% vs 91.7%; p<0.001) and a higher incidence of respiratory-related death (5-year cumulative incidence, 31.1% vs 3.6%; p<0.001), than those without radiological PPFE. In the multivariable analysis, the presence of radiological PPFE was independently associated with all-cause mortality (adjusted HR, 4.78; 95% CI, 2.87 to 7.95; p<0.001) and respiratory-related death (adjusted HR, 3.88; 95% CI, 2.14 to 7.01; p<0.001).InterpretationThis large-scale study demonstrated that in patients with MAC lung disease, radiological PPFE was common, a phenotype associated with unique clinical features and poor prognosis, particularly respiratory-related death. The specific management of this subgroup should be established.

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Section: Discussionmentioning

confidence: 99%

Prognostic significance of radiological pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study

Aono

Hozumi

Kono

et al. 2022

Thorax

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“…As the presenting symptoms of shortness of breath, cough, and chest pain are non-specific, the diagnosis of PPFE is commonly suspected based on the unique findings observed on chest imaging. Chest radiographs show pleural thickening in the upper lobes, with retraction of the hila upwards due to volume loss, with subpleural fibrosis consisting of bands of fibrous tissue extending from the pleura to the hilum [ 4 , 7 , 16 , 23 ]. In addition to pleural thickening and subpleural fibrotic changes, pneumothoraxes may also be seen on chest imaging at the time of presentation or during the disease course [ 8 , 12 , 14 , 18 ].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…On chest CT imaging, pleural thickening with bands of connective tissue directed toward the hilum are described. Although initially described as an upper lobe disease, the lower lobes can also be involved in PPFE, but less commonly [ 4 , 9 , 11 , 23 ]. When lower lobes are involved, an equal distribution between the upper and lower lobes can be observed in up to one third of the patients [ 24 ].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…The initial patients described with PPFE had a diagnosis confirmed via histologic findings, with similar findings seen on lung biopsy or at the time of autopsy [ 5 , 7 , 10 , 11 ]. However, patients with PPFE are at high risk for pneumothorax during their disease course and this is particularly true after undergoing lung biopsy, wherein a persistent pneumothorax may occur, leading to increased morbidity and mortality [ 8 , 12 , 14 , 23 ]. In addition to a pneumothorax as a complication after undergoing a lung biopsy, there is a risk for an acute exacerbation of the PPFE with the development of acute respiratory failure, possibly due to the presence of concomitant UIP [ 8 ].…”

Section: Challenges With Establishing a Diagnosismentioning

confidence: 99%

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Pleuroparenchymal Fibroelastosis: A Review with a Focus on a Non-Infectious Complications after Hematopoietic Stem Cell Transplant

Arndt

2023

Biomedicines

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Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that is currently classified as an idiopathic interstitial pneumonia. Although originally described as an idiopathic disease, PPFE has now been identified as a rare complication following hematopoietic stem cell transplant (HSCT). Unlike other pulmonary complications after HSCT, PPFE occurs very late after transplant. Etiologies for PPFE after HSCT remain to be fully established. Infections and adverse effects to alkylating chemotherapy have been suggested as possible causes. In several cases, there is an association of PPFE with bronchiolitis obliterans syndrome after HSCT, suggesting that PPFE may be another manifestation of pulmonary chronic graft versus host disease after HSCT. Algorithms have been designed to assist in confirming a diagnosis of PPFE without the need for a surgical lung biopsy, however at present, no biomarker is established for the diagnosis or to predict the progression of disease. Presently, there is no current therapy for PPFE, but fortunately the disease progresses slowly in most patients.

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“…Importantly, the prognosis of patients with iPPFE has been reported to be equal to or worse than that of patients with IPF [3,4]. Although antifibrotic therapy has been used for patients with iPPFE, and may slow disease progression, its efficacy has not yet been determined [5][6][7][8]. Further, there are currently no curative treatments for patients with iPPFE.…”

Section: Introductionmentioning

confidence: 99%

Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis

et al. 2023

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BackgroundIdiopathic pleuroparenchymal fibroelastosis (iPPFE) is characterised by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma, with advanced cases often complicated by progressive weight loss. Therefore, we hypothesized that nutritional status is associated with mortality in iPPFE.MethodsThis retrospective study assesses nutritional status at the time of diagnosis and one year after diagnosis in 131 patients with iPPFE. Malnutrition-related risk was evaluated using the Geriatric Nutritional Risk Index (GNRI).ResultsOf the 131 patients, 96 (76.3%) were at malnutrition-related risk at the time of diagnosis according to GNRI. Of these, 21 patients (16.0%) were classified as at major malnutrition-related risk (GNRI <82). Patients at major malnutrition-related risk were significantly older and had worse pulmonary function than patients at low (92≤ GNRI <98)- and moderate (82≤ GNRI <92)-malnutrition-related risk. GNRI scores decreased significantly from the time of diagnosis to one year after diagnosis. Patients with lower GNRI (<91.7) had significantly shorter survival than patients with a median GNRI or higher (≥91.8). Patients with declines in annual GNRI scores of 5 or greater had significantly shorter survival than patients with declines in GNRI scores of less than 5. In multivariate analysis, major malnutrition-related risk was significantly associated with increased mortality after adjustment for age, sex and forced vital capacity (hazard-ratio, 1.957). A composite scoring model including age, sex, and major malnutrition-related risk was able to separate mortality risk in iPPFE.ConclusionAssessment of nutritional status by GNRI provides useful information for managing patients with iPPFE by predicting mortality risk.

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Pleuroparenchymal fibroelastosis

Cited by 23 publications

References 72 publications

Prognostic significance of radiological pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study

Prognostic significance of radiological pleuroparenchymal fibroelastosis inMycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study

Pleuroparenchymal Fibroelastosis: A Review with a Focus on a Non-Infectious Complications after Hematopoietic Stem Cell Transplant

Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis

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