Idiopathic pleuroparenchymatous fibroelastosis: a case report

Fibroelastosis pleuroparenquimatosa idiopática: reporte de caso

Published 2018-05-10

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Vol. 29 No. 2 (2017)
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Presentación de casos
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Idiopathic pleuroparenchymatous fibroelastosis: a case report.
rev. colomb. neumol. [Internet]. 2018 May 10 [cited 2024 Nov. 24];29(2):62-6. Disponible en: https://doi.org/10.30789/rcneumologia.v29.n2.2017.268
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https://doi.org/10.30789/rcneumologia.v29.n2.2017.268
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Tatiana Suárez Poveda
Médica Radióloga. Especialista en Radiología de Trauma y Urgencias. Hospital Universitario San Vicente Fundación.
    Patricia Eugenia Gil Serrano
    Médica Residente de Radiología, Universidad CES. Medellín, Colombia
      María Camila Arango
      Médica Residente de Radiología, Universidad de Antioquia. Medellín, Colombia.
        Viana Palacio
        Médica Residente de Radiología, Universidad de Antioquia. Medellín, Colombia.
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          Idiopathic pleuroparenchymatous fibroelastosis is a recently reported cause of rapidly evolving pulmonary fibrosis and poor prognosis. We describe the case of a previously healthy, young patient who presented in our institution with ventilatory failure and images compatible with pulmonary fibrosis. Pulmonary lobectomy was performed, and histopathological studies confirmed idiopathic pleuroparenchymal fibroelastosis. The patient died before bilateral lung transplant was performed. Clinical, imaginologic, and histopathologic presentation of our case of idiopathic pleuroparenchymal fibroelastosis was usual, chest radiograph and high resolution computed tomography showed volume loss, upper lobes fibrocicatricial changes, pleural and interlobular septal thickening. Pathology showed extensive pleural thickening with inflammatory lymphocytic infiltrate. In conclusion, idiopathic pleuroparenchymal fibroelastosis is a recently described fibrosing lung disease, with poor prognosis, and without standardized diagnostic criteria and treatment. This is why it is important to differentiate it from other interstitial lung diseases.

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