Lung Transplantation for Pleuroparenchymal Fibroelastosis

Shiiya, Haruhiko; Sato, Masaaki

doi:10.3390/jcm10050957

Cited by 10 publications

(12 citation statements)

References 60 publications

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“…However, the survival of patients with radiological PPFE waiting for LT was reported to be non‐inferior to that of patients with fibrotic ILDs without radiological PPFE 17 or those with other IIPs 34 . Recently, a few reports have shown the overall survival and physical outcome of patients with PPFE after LT 35–37 . Our study revealed acceptable post‐LT survival in patients with advanced PPFE, suggesting the suitability of LT for them (both patients with idiopathic disease and patients with secondary disease).…”

Section: Discussionmentioning

confidence: 53%

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: 53%

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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“…Pneumothorax is one of the most common complications in idiopathic and non-idiopathic PPFE. PPFE patients have a higher incidence of pneumothorax than other IIPs [5]. The latency period of PPFE during which patients have no symptoms can be long, and abnormalities would only be noticed in radiological investigations.…”

Section: Discussionmentioning

confidence: 97%

“…In 2013, PPFE was recognized as one of the two most rare idiopathic interstitial pneumonias (IIPs) along with idiopathic lymphoid interstitial pneumonia [ 4 ]. PPFE comprises 5.8% to 10.4% of idiopathic pulmonary fibrosis (IPF) cases [ 5 ]. Oda et al found that 11 of 110 patients (10%) diagnosed with IPF had radiological PPFE while nine (8.2%) had the histological features of PPFE [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

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Pleuroparenchymal Fibroelastosis: A Case Report

Haloui¹,

Allam²,

Nabou³

et al. 2022

Cureus

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Pleuroparenchymal fibroelastosis (PPFE) is a rare, idiopathic interstitial pneumonia. wherein the first symptom might be dyspnea or a dry cough. The condition can also be manifested with chest pain secondary to pneumothorax. While the definitive diagnosis is based on a histological evaluation (which is not often performed), a computed tomography scan shows findings, such as apical fibrosis and pleural thickening of the apical lobes, which help assess the diagnosis. We describe a case of PPFE diagnosed radiologically in a 69-year-old man. This case highlights that PPFE is a pathology that can go unnoticed for a long time, and patients might neglect the revealing symptoms such as coughing.

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“…Lung transplantation is considered the only definitive therapy, but must be considered carefully given the significant pleural involvement that can produce surgical challenges. 7…”

Section: Clinical Discussionmentioning

confidence: 99%

Progressive Dyspnea With Recurrent Pneumothoraces

Homer

Mandziuk

Hirji

et al. 2021

Chest

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A 34-year-old previously healthy man of Korean descent (height, 174 cm; weight, 47.4 kg) demonstrated dyspnea with cough and chest tightness. The patient had no relevant occupational exposures and no history of illicit drug or tobacco use. His medical history was notable for chronic sinus tachycardia of undetermined cause, hypertension, gout, glaucoma of the right eye, and a remote history of an intracranial malignancy 24 years prior treated with unspecified chemotherapy, craniotomy, and ventriculoperitoneal shunt placement. His active medications included diltiazem, candesartan, and colchicine as needed.

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Lung Transplantation for Pleuroparenchymal Fibroelastosis

Cited by 10 publications

References 60 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

Pleuroparenchymal Fibroelastosis: A Case Report

Progressive Dyspnea With Recurrent Pneumothoraces

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