Abstract Introduction Pleural Parenchymal Fibroelastosis (PPFE) is a rare Idiopathic Interstitial Pneumonia (IIP) in which injury-induced reactive fibrosis triggers distortion of lung anatomy. Poor outcomes warrant timely diagnosis and recognition of rare complications, including vocal cord paralysis, with only five documented cases worldwide. The case series describes the only two documented cases in the US. Case 1 A 76-year-old woman with a history of psoriasis, undifferentiated connective tissue disease (UCTD), and fibrosing alopecia presented with chronic dyspnea and dry cough. She was a nonsmoker with no significant environmental or occupational exposures. Chest CT demonstrated fibrotic interstitial lung disease (ILD) with apical predominance, suspicious for PPFE. PFTs showed a mild restrictive defect and a moderate reduction in diffusion capacity. Her ILD was attributed to her UCTD or interstitial pneumonia with autoimmune features. She was initiated on mycophenolate mofetil. Approximately 1.5 years after symptom onset, she developed dysphonia. Laryngoscopy revealed left-sided vocal cord paralysis, likely secondary to upper-lobe-predominant fibrosis. Shortly thereafter, she reported dysphagia, and manometry showed hypercontractile esophagus. Despite treatment, her dyspnea progressed. Follow-up PFTs and imaging revealed advancing fibrosis, prompting the addition of nintedanib. She experienced recurrent hospitalizations for ILD exacerbations with associated functional decline and worsening hypoxia. Serial imaging continued to demonstrate advancing pulmonary fibrosis. She ultimately transitioned to hospice. Case 2 A 55-year-old woman with a history of systemic lupus erythematosus on prednisone and hydroxychloroquine, Mycobacterium avium-intracellulare infection treated with triple therapy, and pulmonary embolism (PE) was evaluated following hospitalization for hemoptysis. Imaging revealed bilateral upper zone predominant reticulation, fibrosis, and traction bronchiectasis. Bronchoscopy was unrevealing. Over the subsequent months, she experienced progressive dyspnea and recurrent hospitalizations for COVID-19, Pseudomonas pneumonia, acute subsegmental PE, and pneumothorax. Serial chest CT scans demonstrated progression of mid-to-upper lung-predominant subpleural consolidation, reticulation, and traction bronchiectasis, raising suspicion for PPFE. She developed dysphagia and dysphonia with esophagram demonstrating esophageal dysmotility. Cryobiopsy of the lung was performed; pathology suggested PPFE with possible vagal/recurrent laryngeal nerve involvement. She was initiated on nintedanib. Ultimately, she continued to decline and elected to pursue hospice. Discussion PPFE is a rare IIP, and VCP is an exceedingly rare complication. This case series highlights the variable presentation and progression of PPFE, suggesting that inflammatory lung damage may induce adjacent anatomic changes, leading to the insidious onset of nerve-mediated VCP and esophageal dysmotility. Given the significant morbidity, early screening and symptomatic management may improve outcomes and slow disease progression. This abstract is funded by: None
Eltarras et al. (Fri,) studied this question.