Abstract Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) resulting from repeated exposure to an inhaled antigen, leading to inflammatory and/or fibrotic lung disease. Diagnosis can be challenging due to the insidious onset of respiratory symptoms and the fact that 15-50% of HP cases remain antigen-indeterminate. The progression from acute/subacute HP to a chronic fibrosing phenotype is variable but commonly occurs over several years. We present a case of a patient with no known pulmonary disease who developed rapidly progressive fibrotic ILD after parakeet exposure, complicated by recurrent pneumothoraces, effectively managed by bronchoscopic endobronchial valve (EBV) placement. A 39-year-old female with a 5-pack-year tobacco history presented with cough and dyspnea. CT imaging revealed extensive bilateral ground-glass opacities and emphysema. Initially diagnosed with COVID-19 pneumonia, she developed a spontaneous secondary pneumothorax one month later, successfully treated with chest tube placement. Six weeks after, she re-presented with dyspnea; CT demonstrated bilateral pneumothoraces, interstitial thickening, bronchiectasis, and cystic changes, highly suggestive of rapidly progressing fibrosis. Bilateral chest tubes were inserted, and persistent air leaks indicated presence of bronchopleural fistulas. Given the severity of her pulmonary fibrosis, surgical procedures were deferred. Therefore, bilateral EBVs were placed bronchoscopically, successfully resolving air leaks and improving pneumothoraces. Autoimmune serologies were negative. The patient was empirically treated with steroids but diagnosis remained elusive as parakeet exposure had not yet been uncovered. She later returned to the hospital with a fourth spontaneous pneumothorax. A thorough history review unveiled chronic parakeet exposure during the year prior to symptom onset. The bird had since been rehomed. She subsequently underwent a right-sided VATS lung biopsy, which revealed poorly formed granulomas and airway-centered ILD, consistent with fibrotic HP. The VATS biopsy was complicated by a persistent post-procedural pneumothorax and bronchopleural fistula, which was again successfully treated via EBVs. She remains oxygen-dependent while on a steroid taper and has been initiated on the antifibrotic medication nintedanib. She is currently being considered for lung transplant. This case illustrates the potential for rapid progression of fibrotic HP, suggesting a possible genetic predisposition to robust immune response in certain individuals despite antigen removal. EBVs provided a crucial, less invasive alternative to surgery for managing persistent air leaks and recurrent pneumothoraces in a patient whose advanced fibrotic lung disease rendered her a high-risk candidate for traditional surgical interventions. EBVs helped stabilize her respiratory status and facilitated bridging to antifibrotic therapy and transplant consideration. This abstract is funded by: None
Touloumes et al. (Fri,) studied this question.