CHEST Guidelines-Progressive-Dyspnea-With-Recurrent-Pneumothoraces

Progressive-Dyspnea-With-Recurrent-Pneumothoraces_

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The case report discusses a 34-year-old Korean man with progressive dyspnea and recurrent pneumothoraces, leading to a diagnosis of idiopathic pleuroparenchymal fibroelastosis (PPFE), a rare type of interstitial pneumonia. Initially presenting with dyspnea, cough, and low arterial oxyhemoglobin saturation, the man experienced recurrent hospital admissions due to pneumothoraces over nearly two years. Despite treatment with pigtail catheter placements, his condition worsened, necessitating urgent surgery for a right thoracoscopic bullectomy, pleurectomy, and pleurodesis. However, complications followed, including significant hypoxia and aspiration during the perioperative period, which required reintubation and intensive care, eventually leading to his listing for a lung transplant. He received venovenous extracorporeal membrane oxygenation therapy for five weeks until donor lungs became available, and underwent a bilateral orthotopic sequential lung transplantation, from which he recovered uneventfully.<br /><br />Diagnostic imaging initially showed bilateral fibrotic changes and pneumothoraces, while high-resolution CT scans later revealed pleural and lung parenchymal abnormalities, indicative of PPFE. Pathological analysis of the explanted lungs highlighted extensive pleural fibrosis and fibroelastosis, predominantly affecting the upper lobes but also involving lower lobes, challenging typical PPFE distribution patterns that are usually restricted to upper lobes.<br /><br />PPFE is characterized by progressive fibrosis and elastosis in lung subpleural areas and has associations with previous treatments such as chemotherapy or conditions like hypersensitivity pneumonitis. Its diagnosis is crucial due to the progressive nature, with limited therapeutic options—namely corticosteroids, antifibrotic therapies like nintedanib, and lung transplantation representing definitive interventions. The case underscores the importance of early diagnosis and the potential need for lung transplantation in severe cases, especially in younger patients.

Keywords

idiopathic pleuroparenchymal fibroelastosis

interstitial pneumonia

pneumothoraces

lung transplantation

extracorporeal membrane oxygenation

thoracoscopic bullectomy

pleurodesis

fibrotic changes

corticosteroids

antifibrotic therapies