Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients

Mariani, Francesca; Gatti, B; Rocca, Alberto; Bonifazi, Francesca; Cavazza, Alberto; Fanti, Stefano; Tomassetti, Sara; Piciucchi, Sara; Poletti, Venerino; Zompatori, Maurizio

doi:10.5152/dir.2016.15516

Cited by 67 publications

(65 citation statements)

References 36 publications

(11 reference statements)

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“…In terms of RV/TLC, Oda et al [16] suggested that, despite upper lobe volume loss, RV/TLC was higher in PPFE patients than in other IIP patients because RV was relatively more increased than TLC owing to compensatory hyperinflation of the middle and lower lobes. PPFE could be related to relevant underlying conditions including infection, drugs, autoimmune disease, and organ transplantation, and the reports of PPFE after organ transplantation have increased [23][24][25][26]. In our study, among 26 patients, two patients (7.7%) had a history of organ transplantation (one heart and one kidney), consistent with previous results; in the study including 12 patients with PPFE, Reddy et al [3] reported that one patient (8.3%) had a history of kidney transplantation.…”

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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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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“…Mariani et al retrospectively reviewed CT scans of 700 HSCT and 53 lung transplant recipients to assess the prevalence of PPFE (based on radiographic criteria) in these patient populations. They reported a 0.28% prevalence in HSCT recipients and a 7.54% prevalence in lung transplant recipients 10. Other possible aetiologies of secondary PPFE that have been reported in the literature include cyclophosphamide, carmustine and aluminosilicate dust exposure 11–13.…”

Section: Discussionmentioning

confidence: 99%

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

Ali

Ramalingam²,

Haasler

et al. 2019

BMJ Case Rep

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A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Non-invasive workup was non-revealing; therefore, the patient was referred for video-assisted thoracic surgery and lung biopsy. Histopathology revealed pleural thickening and, subpleural parenchymal fibrosis and elastic tissue deposition. Lung parenchyma further away from the pleura was well preserved. Based on these findings, the patient was diagnosed with pleuroparenchymal fibroelastosis (PPFE). Since PPFE is a progressive disorder without effective medical therapies, and given our patient’s worsening symptoms, she underwent bilateral lung transplantation. It has been almost 4 years since the lung transplantation, our patient continues to do well. To the best of our knowledge, to date, this is the longest follow-up reported for a PPFE patient undergoing lung transplantation.

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“…The disease may be idiopathic or associated with other disorders [4]. Some patients may develop PPFE after lung, bone marrow or hematopoietic cell transplant [5], immunosuppressive treatment [6] or occupational exposure to silicate dust and asbestos [7,8]. A history of recurrent lower respiratory tract infections is also common, suggesting that repeated inflammatory stimuli may contribute to the development of PPFE [9].…”

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confidence: 99%

“…Common symptoms of PPFE are dyspnea with insidious onset, dry cough, and weight loss; during the course of the disease, some patients can develop spontaneous pneumothorax [5,16]. As a rule, PPFE is associated with restrictive functional deficit and a decrease in carbon monoxide diffusion capacity [5].…”

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confidence: 99%

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Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

Bargagli¹,

Rottoli²,

Torricelli³

et al. 2018

Pathobiology

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Pleuroparenchymal fibroelastosis is a rare form of upper-lobe-dominant progressive pulmonary fibrosis characterized histologically by visceral pleural thickening with collagenous fibrosis, subpleural elastosis, and intra-alveolar collagenous fibrosis. It was first described 25 years ago by Amitani et al. This report firstly describes a new variant or rare phenotype of PPFE with airway involvement, minimal pleuroparenchymal connections, and non-necrotizing granulomas.

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Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients

Cited by 67 publications

References 36 publications

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

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

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

Airway-Centered Pleuroparenchymal Fibroelastosis Associated with Non-Necrotizing Granulomas: A Rare New Entity

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