CHEST Guidelines-Chronic-Progressive-Dyspnea-in-a-71-Year-Old-Man

Chronic-Progressive-Dyspnea-in-a-71-Year-Old-Man_c

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A 71-year-old former bus driver and smoker presented with chronic progressive dyspnea and dry cough, symptoms that began insidiously eight years prior. He also reported significant weight loss and proximal muscle weakness. His medical history includes a gastric adenocarcinoma diagnosis followed by surgery and chemotherapy, as well as heart disease treated with coronary bypass surgery.<br /><br />Over the years, numerous diagnostic procedures had been performed with inconclusive results. Upon his latest hospital visit, physical examinations revealed severe emaciation and muscle atrophy but no finger clubbing or abnormal heart sounds. Lung auscultation showed fine crackles at the lung bases. Laboratory tests indicated hypoxemia and a restrictive defect in lung function. Chest imaging showed irregular pleural thickening, subpleural airspace consolidation, and bronchiectasis, predominantly affecting the upper lobes.<br /><br />The condition was eventually diagnosed as pleuroparenchymal fibroelastosis (PPFE), a rare lung disease characterized by fibrosis, mostly affecting the upper lung lobes. The condition can be idiopathic or related to factors such as pneumonia, cancer treatments, and other conditions. Its diagnosis involved correlating clinical, radiologic, and histologic characteristics, given the absence of definitive guidelines. Histological examination confirmed dense fibrosis and alveolar septal elastosis.<br /><br />PPFE often presents with symptoms like dyspnea, cough, and thoracic cage changes. Lung function tests typically show a restrictive defect, and the disease can progress at varying rates, often without effective treatment options aside from lung transplantation.<br /><br />In this case, despite comprehensive supportive care, the patient succumbed to the disease shortly after diagnosis, underlining the aggressive nature of PPFE and the limitations in current treatment strategies. The case highlights the importance of considering PPFE in patients with similar clinical presentations and the potential role of past treatments or conditions in its development.

Keywords

pleuroparenchymal fibroelastosis

lung fibrosis

dyspnea

chronic cough

restrictive lung defect

hypoxemia

gastric adenocarcinoma

coronary bypass surgery

emaciation

bronchiectasis