Abstract Introduction Pleuroparenchymal fibroelastosis (PPFE) is a rare progressive interstitial lung disease (ILD) characterized by upper lobe pleural and subpleural fibrosis. Most common form is idiopathic; however, it has also been associated with connective tissue disease, post-lung transplant rejection, and chronic graft vs host disease post-bone marrow transplant. PPFE typically results in restrictive pulmonary dysfunction and progressive respiratory failure, frequently complicated by malnutrition, platythorax, and recurrent pneumothoraces. Case Presentation A 57-year-old male presented with chronic shortness of breath, right-sided chest pain, and over 40 lbs of weight loss over two years. In 2018, he developed dyspnea on exertion; findings on imaging revealed bilateral apical and parenchymal opacities, more on the right. By July 2024, disease progression included severe upper lobe fibrosis, volume loss, and architectural distortion. Bilateral pleural effusions (now more worse on the left), small pneumothoraces, and a left pneumothorax extending anterior to the heart were noted. After multiple ED visits, the patient was admitted in February 2025 due to a new extensive pneumomediastinum dissecting into the neck, following several ED visits. Past medical history Smoker (7.1 pack-years), history of marijuana use, right-sided stab wound without lung penetration. Clinical course The patient was admitted to the hospital, and the main focus was supportive care, including oxygen therapy. A lung transplant evaluation was started; however, the patient was not deemed a candidate at that time due to comorbidities and malnutrition. He eventually passed away in a subsequent hospital stay for pneumonia. Conclusions PPFE is a rare progressive ILD, with upper lobe predominant and subpleural disease on Chest CT. Disease course is often indolent, with frequent episodes of pneumothoraces and pneumomediastinum. With no effective medical treatment available, early referral for lung transplant evaluation is recommended. References 1.Ishii H, Kinoshita Y, Hamada N, Fujita M, Kushima H. Idiopathic pleuroparenchymal fibroelastosis: diagnosis and management. Expert Rev Respir Med. Published online May 5, 2025. doi:10.1080/17476348.2025.2499651 2. Suzuki Y. Idiopathic pleuroparenchymal fibroelastosis: A review of the previous literature and current knowledge. Respir Investig. 2025;63(1):127-137. doi:10.1016/j.resinv.2024.12.009 This abstract is funded by: None
Verma et al. (2026) studied this question.