Pleuroparenchymatous fibroelastosis and pulmonary hypertension: a different phenotype?
DOI:
https://doi.org/10.53732/rccsalud/2023.e5208Keywords:
lung disease, interstitial, pulmonary fibrosis, pleural diseases, pleuroparenchymal fibroelastosisAbstract
Pleuroparenchymal fibroelastosis is an unusual lung disease with unique clinical, radiological, and pathological characteristics that has recently been included in the updated consensus on idiopathic interstitial pneumonia. Their name refers to a combination of fibrosis involving the visceral pleura and fibro-elastic changes that predominate in the subpleural pulmonary parenchyma. Although several associations between diseases have been described, no single cause has been unequivocally identified. The diagnosis is based on clinical-radiological criteria and histopathological findings when biopsies are available. There are few reports on the association between this entity and pulmonary hypertension. There is no treatment to date, although some cases of lung transplantation have been successful. We describe the clinical characteristics of a patient treated in a hospital in Paraguay.
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