Isolated Left Lower Lobe Pleuroparenchymal Fibroelastosis Presenting as Hemothorax.

Abstract

Characterized by pleural and subpleural fibrosis with alveolar septal elastosis, pleuroparenchymal fibroelastosis is a rare restrictive lung disease. Symptoms are often subtle, including dyspnea, cough, and weight loss; while acute presentations of spontaneous pneumothorax have been recorded. We report a patient who developed a spontaneous hemothorax, who upon evacuation of the chest was found to have a hemorrhagic lower lobe mass consistent with pleuroparenchymal fibroelastosis. Various conditions are associated with pleuroparenchymal fibroelastosis, suggesting chronic lung injury as a factor in pathogenesis. Hemothorax of this magnitude with relatively no known inciting risk factors, represents an exceptionally rare case presentation.